Anti-ILT7 binding agents for the treatment and prevention of myositis

Abstract

The present disclosure relates to compositions and methods for treating diseases (e.g., myositis diseases and disorders) in a subject in need thereof using anti-ILT7 antibodies or antigen-binding fragments thereof. Immunoglobulin-like transcript 7 (ILT7) can be targeted for the treatment and prevention of myopathy. Accordingly, provided are compositions and methods of using anti-ILT7 antibodies or antigen-binding fragments thereof to reduce or eliminate symptoms or diseases associated with ILT7.

Description

[Technical Field]

[0001] CROSS-REFERENCE TO RELATED APPLICATIONS This application claims priority to U.S. Patent Application No. 63 / 387,023, filed December 12, 2022, the contents of which are incorporated herein by reference in their entirety for all purposes.

[0002] Sequence Listing Reference The contents of the electronic sequence listing (HOPA_062_01WO_SeqList_ST26.xml; size: 10,663 bytes; and creation date: December 4, 2023) are incorporated herein by reference in their entirety.

[0003] The present disclosure relates to compositions comprising ILT7 binding proteins and methods of using such compositions in the treatment and prevention of myositis. [Background technology]

[0004] If idiopathic inflammatory myopathy is not promptly controlled, it can lead to debilitating and permanent muscle damage. Currently, corticosteroids, intravenous immunoglobulin (IVIG), and other immunomodulatory agents are used for treatment, but these medications are also associated with side effects, including the risk of diabetes, osteoporosis, cataracts, opportunistic infections, and thrombosis. In addition, some subjects do not achieve timely disease control with these therapies. This, combined with the burden of side effects of the therapies, has created a clinical need for therapies to treat these patients.

[0005] Immunoglobulin-like transcript 7 (ILT7) can be targeted for the treatment and prevention of myopathy. Accordingly, provided are compositions and methods of using anti-ILT7 antibodies or antigen-binding fragments thereof to reduce or eliminate symptoms or diseases associated with ILT7. Summary of the Invention [Means for solving the problem]

[0006]

[0013] A method for treating dermatomyositis is provided, comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of such treatment, wherein the ILT7 antibody or antigen-binding fragment thereof comprises complementarity-determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively; the effective amount is about 300 mg; and the antibody is administered once every four weeks. In one embodiment, the dermatomyositis includes definite myositis. In one embodiment, the dermatomyositis includes probable myositis.

[0007] A method for treating anti-synthetase inflammatory myositis (ASIM) is provided, the method comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of treatment for ASIM, wherein the ILT7 antibody comprises complementarity-determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, the effective amount is about 300 mg, and the antibody is administered once every four weeks. In one embodiment, ASIM includes definite myositis. In some embodiments, ASIM includes probable myositis.

[0008] A method for treating a disease is provided, the method comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or an antigen-binding fragment thereof to a subject in need of disease treatment, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, the effective amount comprising a dose of about 300 mg, and the administering is completed once every four weeks.

[0009] Methods for treating a disease are provided, the method comprising administering an effective amount of an anti-Immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of disease treatment, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and wherein the administration is effective to reduce the disease compared to (a) the disease in an otherwise comparable subject lacking said administration, or (b) a baseline measure of the disease in the subject in need of disease treatment.

[0010] In one embodiment, the administering continues for at least about 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, or up to about 15 administrations. In an embodiment, the administering is effective in alleviating disease in a subject in need of disease treatment compared to a baseline measurement of the disease, where alleviation is measured by detecting a decreased total improvement score in the subject. In an embodiment, the administering is effective in alleviating disease in a subject in need of disease treatment compared to a baseline measurement of the disease, where alleviation is measured by detecting a decreased level of disease symptoms in the subject. In an embodiment, the measurement is completed after 6 and / or 12 administrations. In an embodiment, the total improvement score is 10 or more, 20 or more, 30 or more, 40 or more, or 50 or more. In an embodiment, the total improvement score is 20 or more. In an embodiment, the total improvement score is 40 or more. In embodiments, the level of symptom reduction comprises a decrease in a score on a survey selected from the group consisting of the Dermatomyositis Disease Area and Severity Index (CDASI), the Myositis Disease Activity Assessment Tool (MDAAT), the Myositis Damage Index (MDI), and combinations thereof, compared to baseline. In embodiments, the level of symptom reduction comprises an increase in pulmonary compliance, as measured by an increase in forced vital capacity (FVC), compared to baseline. In embodiments, the administering is (b) effective in reducing disease in a subject in need of treatment for the disease, compared to a baseline measure of disease, where reduction is measured by a reduction in corticosteroid use, with measurement completed after six doses. In embodiments, the corticosteroid comprises prednisone. In embodiments, the reduction comprises a decrease in frequency of use of at least about 10%, 15%, 20%, 25%, 30%, or 40%. In embodiments, the reduction is at a dose of about 10 mg / day or more to a dose of 7.5 mg / day. In aspects, the ILT7 antibody comprises a heavy chain variable region (VH) and a light chain variable region (VL), wherein the VH and VL regions comprise amino acid sequences at least 80% identical to SEQ ID NO:2 and SEQ ID NO:7, respectively. In aspects, the ILT7 antibody comprises a heavy chain variable region (VH) and a light chain variable region (VL), wherein the VH and VL regions comprise the amino acid sequences of SEQ ID NO:2 and SEQ ID NO:7, respectively. In aspects, the ILT7 antibody is a monoclonal antibody. In aspects, the ILT7 antibody is non-fucosylated.In embodiments, the subject's sample is positive for one or more ASIM-related antibodies. In embodiments, the one or more ASIM-related antibodies are selected from the group consisting of anti-Jo-1, anti-PL-12, anti-PL-7, anti-KS, anti-EJ, anti-OJ, anti-ZO, and anti-YRS (HA). In embodiments, the subject in need of treatment has a dermatomyositis-associated rash. In embodiments, the disease is caused by plasmacytoid dendritic cells. In embodiments, the administration is effective in reducing the level of type I interferon in the subject compared to the subject's baseline level. In embodiments, the level is reduced by at least about 1-fold, 5-fold, 10-fold, 25-fold, 50-fold, 75-fold, 100-fold, 150-fold, or 250-fold. In embodiments, the administration is effective in eliminating the disease for at least about 3 months, 6 months, 1 year, or 2 years. In embodiments, the administration is subcutaneous.

[0011] A method for treating dermatomyositis is provided, the method comprising subcutaneously administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of treatment for dermatomyositis, wherein the ILT7 antibody or antigen-binding fragment thereof comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and the effective amount is about 300 mg.

[0012] A method for treating anti-synthetase inflammatory myositis (ASIM) is provided, the method comprising subcutaneously administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or an antigen-binding fragment thereof to a subject in need of treatment for ASIM, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and the effective amount is approximately 300 mg.

[0013] These and other aspects are described below.

[0014] The accompanying drawings, which are incorporated in and form a part of this specification, illustrate some, but not the only or exclusive, aspects and / or features. It is intended that the aspects and figures disclosed herein be considered illustrative rather than limiting. [Brief explanation of the drawings]

[0015] [Figure 1] 1 shows an exemplary study flow chart. DETAILED DESCRIPTION OF THE INVENTION

[0016] The present disclosure provides antibodies and antigen-binding fragments thereof that bind to immunoglobulin-like transcript 7 (ILT7). Inhibition of binding can reduce or eliminate activation of inflammatory signaling pathways associated with related diseases. In embodiments, the anti-ILT7 antibodies and antigen-binding fragments disclosed herein can be used to neutralize ILT7. In embodiments, the anti-ILT7 antibodies and antigen-binding fragments disclosed herein can be used to regulate pDC activity. In embodiments, the anti-ILT7 antibodies and antigen-binding fragments disclosed herein can be used to regulate IFN release. In embodiments, the anti-ILT7 antibodies and antigen-binding fragments disclosed herein can be used to reduce or eliminate ILT7 / BST2 binding. Such an approach is advantageous because it attacks inflammation at the signaling source, allowing for more robust anti-inflammatory treatment effects for diseases. Polynucleotides encoding the anti-ILT7 antibodies and antigen-binding fragments, encoded polypeptides, and pharmaceutical compositions comprising the same are provided. Methods of using anti-ILT7 antibodies and antigen-binding fragments thereof in methods of treating diseases are also disclosed herein. In embodiments, anti-ILT7 antibodies and antigen-binding fragments thereof are used in methods of treating dermatomyositis (DM) and / or anti-synthetase inflammatory myositis (ASIM).

[0017] definition Unless otherwise defined, all technical and scientific terms used herein have the same meaning as commonly understood by one of ordinary skill in the art to which the subject matter pertains. All publications, patent applications, patents, and other references mentioned herein are expressly incorporated by reference in their entirety. In case of conflict, the present specification, including definitions, will control. Furthermore, the materials, methods, and examples described herein are merely illustrative and are not intended to be limiting.

[0018] As used in this specification and the appended claims, the singular forms "a," "an," and "the" include plural referents unless the context clearly dictates otherwise.

[0019] The term "about" or "approximately," when immediately preceding a numerical value, denotes a range (e.g., plus or minus 10% of that value). For example, "about 50" can mean 45 to 55, and "about 25,000" can mean 22,500 to 27,500, unless the context of this disclosure dictates otherwise or contradicts such an interpretation. For example, in a list of numerical values ​​such as "about 49, about 50, about 55, ...," "about 50" means a range extending to less than half the interval between the preceding and following values, e.g., greater than 49.5 to less than 52.5. Furthermore, the phrase "less than about" a value or "greater than about" a value should be understood in light of the definition of the term "about" provided herein. Similarly, the term "about" when preceding a series of numerical values ​​or ranges of values ​​(e.g., "about 10, 20, 30," or "about 10 to 30") refers to all values ​​in the series or to the endpoints of the range, respectively.

[0020] As used herein, the term "subject" refers to any subject, e.g., a human or non-human mammal, for whom diagnosis, prognosis, or therapy is desired. The term "subject" can refer to a human or non-human mammal that is suffering from, may be suffering from, or is suspected of being suffering from a disease. The terms "subject" and "subject" are used interchangeably herein. In embodiments, a subject is a mammal. Mammals include primates such as humans, monkeys, chimpanzees, and apes, as well as non-primates such as domestic and wildlife animals, including laboratory animals (such as rabbits and rodents, e.g., guinea pigs, rats, or mice), and domestic pets and livestock (e.g., cats, dogs, pigs, cows, sheep, goats, horses, rabbits), and non-domestic animals such as birds, reptiles, and fish.

[0021] As used herein, the term "subject in need" includes subjects who can or would benefit from the methods described herein. Subjects in need of treatment include, but are not limited to, subjects already with a condition or disorder, subjects prone to having a condition or disorder, subjects suspected of having a condition or disorder, as well as subjects in whom a condition or disorder is to be prevented, ameliorated, or reversed.

[0022] As used herein, "treating" or "treat" describes the management and care of a subject for the purpose of combating a disease, condition, or disorder, and includes administering an anti-ILT7 antibody or antigen-binding fragment thereof used in the methods described herein to alleviate the symptoms or complications of the disease, condition, or disorder, or to eliminate the disease, condition, or disorder. Thus, the term "treat" or "treating" refers to both therapeutic and prophylactic or preventative measures, where the goal is to prevent, slow (alleviate), or ameliorate the progression of a disease (e.g., a myositis disease or disorder). Beneficial or desired clinical results include, but are not limited to, alleviation of symptoms, reduction in the extent of the disease, stabilization (i.e., non-worsening) of the disease, delay or slowing of disease progression, improvement or alleviation of the disease state, and reversal of the disease (whether partial or total). The term "treating" can also include treatment of cells in vitro or in an animal model.

[0023] When referring to nucleic acid or protein sequences, the term "identity" is used to indicate the similarity between two sequences. Unless otherwise indicated, percent identity described herein is determined using the BLAST algorithm, available on the World Wide Web at the following address: blast.ncbi.nlm.nih.gov / Blast.cgi, using default parameters.

[0024] Immunoglobulin-like transcript 7 (ILT7) binding protein Plasmacytoid dendritic cells (pDCs) are key regulators of the immune system and a major source of type I interferons (IFNs, e.g., IFN-α). IFN-α is a key cytokine in the innate response, enhancing activation of all arms of the immune system. pDCs release IFNs in response to nucleic acids sensed by Toll-like receptors (TLRs) TLR7 and TLR9, which are expressed on the surface of pDCs. TLR-induced responses are regulated by receptors containing immunoreceptor tyrosine-based activation motifs (ITAMs). Immunoglobulin-like transcript 7 (ILT7), also known as LIRA4, LILRA4, or CD85g, is one such receptor. ILT7 contains four immunoglobulin-like extracellular domains and one transmembrane domain. The extracellular portion is important for interaction with the ILT7 ligand, bone marrow stromal cell antigen 2 (BST2), and the transmembrane domain of ILT7 contains positively charged residues that enable complex formation with FcεRIγ. BST2-ILT7 interaction negatively regulates the innate immune function of pDCs, where in vitro antibody cross-linking of ILT7 has been shown to negatively regulate the production of IFN-α and TNF-α by pDCs.

[0025] Provided herein are compositions that bind to ILT7. In embodiments, provided compositions comprise an ILT7 antagonist. In embodiments, provided compositions bind to ILT7 and neutralize ILT7 activity. In embodiments, provided compositions comprise an immunoglobulin G (IgG) human monoclonal antibody (mAb). In embodiments, provided herein are compositions that bind to ILT7 and have antibody-dependent cellular cytotoxicity (ADCC) activity. In embodiments, provided herein are compositions comprising an anti-ILT7 antibody or antigen-binding fragment thereof.

[0026] In embodiments, anti-ILT7 antibodies include antibodies that bind to ILT7. ILT7 binding can be mediated by any portion of the antibody. In embodiments, the antibody or antigen-binding fragment binds to ILT7 through a complementarity-determining region (CDR). In embodiments, the antibody binds to ILT7 through the VL and / or VH chain. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof comprises six complementarity-determining regions (CDRs), referred to as heavy CDR1 (HCDR1), HCDR2, HCDR3, light CDR1 (LCDR1), LCDR2, and LCDR3. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof comprises two light chain variable loop domains (VL) and two heavy chain variable loop domains (VH). Basic antibody structure in vertebrate systems is relatively well understood. See, e.g., Harlow et al., Antibodies: A Laboratory Manual, (Cold Spring Harbor Laboratory Press, 2nd ed., 1988). Antibodies or antigen-binding fragments, variants or derivatives thereof include, but are not limited to, polyclonal, monoclonal, human, humanized, or chimeric antibodies, epitope-binding fragments such as Fab, F(ab'), Fv, single-chain Fv (scFv), single-chain antibodies, disulfide-linked Fv (sdFv), fragments comprising either the VL or VH domain (fd), fragments produced by a Fab expression library, and other antibody fragments and combinations thereof that retain antigen-binding function, e.g., the ability to bind ILT7.

[0027] In aspects, the anti-ILT7 antibody, or antigen-binding fragment thereof, comprises a VL domain and a VH domain, wherein the VH domain comprises the polypeptide of SEQ ID NO: 2 and the VL domain comprises the polypeptide of SEQ ID NO: 7. In aspects, the anti-ILT7 antibody comprises two VL domains and two VH domains, wherein the VH domain comprises the polypeptide of SEQ ID NO: 2 and the VL domain comprises the polypeptide of SEQ ID NO: 7. In aspects, the nucleic acid sequences of the VH and VL domains comprise or consist of nucleic acid SEQ ID NO: 1 and nucleic acid SEQ ID NO: 6, respectively.

[0028] In aspects, the anti-ILT7 antibody or antigen-binding fragment thereof comprises six CDR regions designated HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1 comprises the amino acid sequence of SEQ ID NO: 3, HCDR2 comprises the amino acid sequence of SEQ ID NO: 4, HCDR3 comprises the amino acid sequence of SEQ ID NO: 5, LCDR1 comprises the amino acid sequence of SEQ ID NO: 8, LCDR2 comprises the amino acid sequence of SEQ ID NO: 9, and LCDR3 comprises the amino acid sequence of SEQ ID NO: 10.

[0029] Exemplary sequences of anti-ILT7 antibodies and antigen-binding fragments thereof are shown in Table 1. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof comprises at least, or at most, about 50%, 55%, 60%, 65%, 70%, 75%, 80%, 85%, 90%, 95%, 96%, 97%, 98%, 99%, or up to about 100% identity to any one of SEQ ID NOs: 1-10 set forth in Table 1. In embodiments, any one of the sequences in Table 1 can be modified. In embodiments, modifications include one or more truncations, deletions, insertions, and combinations thereof. Modifications can occur at any of the residues provided in Table 1 and at any number of residues from Table 1. In embodiments, modifications can include 1 to 3, 1 to 5, 1 to 10, 1 to 20, 3 to 8, 3 to 10, 3 to 15, 5 to 8, 5 to 10, or 5 to 20 residues. In aspects, modifications can occur at no more than 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 20, 30, 40, 50, 60, 70, 80, 90, 100, 200, or 300 residues.

[0030] [Table 1]

[0031] In some embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof of the present disclosure can include sequence variants as long as they retain the ability to bind to ILT7. For example, conservative amino acid substitutions can be made within any one of the disclosed CDRs. Various changes can be made according to the methods disclosed in Antibody Engineering, 2nd ed., Oxford University Press, ed. Borrebaeck, 1995. These changes include, but are not limited to, nucleotide sequences that are altered by substitution of different codons encoding functionally equivalent amino acid residues within the sequence, thus producing "silent" changes. For example, nonpolar amino acids include alanine (A), leucine (L), isoleucine (I), valine (V), proline (P), phenylalanine (F), tryptophan (W), and methionine (M). Polar neutral amino acids include glycine (G), serine (S), threonine (T), cysteine ​​(C), tyrosine (Y), asparagine (N), and glutamine (Q). Positively charged (basic) amino acids include arginine (R), lysine (K), and histidine (H). Negatively charged (acidic) amino acids include aspartic acid (D) and glutamic acid (E). In embodiments, the CDR amino acid sequences disclosed herein (SEQ ID NOS: 3-5, 8-10) can vary by at least about 80%, 85%, 90%, 95%, 96%, 97%, 98%, 99%, or up to about 100%.

[0032] Derivatives and analogs of antibodies or antigen-binding fragments thereof of the present disclosure can be produced by a variety of techniques, including recombinant and synthetic methods (Maniatis (1990) Molecular Cloning, A Laboratory Manual, 2nd ed., Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, and Bodansky et al. (1995) The Practice of Peptide Synthesis, 2nd ed., Spring Verlag, Berlin, Germany). In an aspect, a method for producing a VH domain that is an amino acid sequence variant of a VH domain of the present disclosure comprises adding, deleting, substituting, or inserting one or more amino acids into the amino acid sequence of a VH domain disclosed herein, optionally combining the resulting VH domain with one or more VL domains, and testing the VH domain or VH / VL combination(s) for specific binding to an antigen. Similar methods can be used to combine one or more sequence variants of a VL domain disclosed herein with one or more VH domains. In embodiments, the amino acid and / or nucleic acid VH or VL sequences disclosed herein (SEQ ID NOS: 1, 2, 6, and 7) can vary by at least about 80%, 85%, 90%, 95%, 96%, 97%, 98%, 99%, or up to about 100%.

[0033] The present disclosure provides methods of using immunoglobulin-like transcript 7 (ILT7) binding molecules, e.g., antibodies and antigen-binding fragments thereof that bind to ILT7. The full-length amino acid and nucleotide sequences for ILT7 are known in the art (e.g., UniProt Acc. No. P59901 for human ILT7). ILT7-specific antibodies or antigen-binding fragments of the present disclosure inhibit BST2-mediated activation of ILT7, thereby reducing proinflammatory signaling and alleviating inflammation in a subject. In embodiments, the anti-ILT7 antibody is also known as HZN-7734. In embodiments, the anti-ILT7 antibody is also known as MEDI7734. In embodiments, the anti-ILT7 antibody is also known as VIB7734. In embodiments, the anti-ILT7 antibody is also known as daxudilimab. In embodiments, the compositions of the present disclosure can include any of the amino acid or nucleic acid sequences described in U.S. Pat. No. 11,072,652, the entire contents of which are incorporated herein by reference. In embodiments, the compositions provided can include any of the sequences shown in Table 1.

[0034] In embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof disclosed herein can be glycosylated. In embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof can have a glycosylation pattern that differs from the wild-type pattern. For example, one or more carbohydrate moieties can be deleted and / or one or more glycosylation sites can be added. Adding glycosylation sites to the antibodies or antigen-binding fragments disclosed herein can be accomplished by altering the amino acid sequence to include glycosylation site consensus sequences well known in the art. Another method for increasing the number of carbohydrate moieties on an antibody or antigen-binding fragment is by chemically or enzymatically coupling glycosides to amino acid residues of the antibody or antigen-binding fragment. Such methods are described in WO 87 / 05330 and Aplin et al. (1981) CRC Crit. Rev. Biochem., 22:259-306. Removal of any carbohydrate moieties from the antibody or antigen-binding fragment can be accomplished chemically or enzymatically, for example, as described in Hakimuddin et al. (1987) Arch. Biochem. Biophys., 259:52; and Edge et al. (1981) Anal. Biochem., 118:131, and Thotakura et al. (1987) Meth. Enzymol., 138:350.

[0035] In embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof disclosed herein can be nonfucosylated. In embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof can have a fucosylation pattern that differs from the wild-type pattern. For example, one or more fucose moieties can be deleted. Deletion of fucosylation sites from the antibodies or antigen-binding fragments disclosed herein can be achieved by altering the amino acid sequence to remove fucosylation site consensus sequences well known in the art. Alternative means of removing any fucose moieties from antibodies or antigen-binding fragments can be achieved chemically or enzymatically, e.g., as described in Hakimuddin et al. (1987) Arch. Biochem. Biophys., 259:52; and Edge et al. (1981) Anal. Biochem., 118:131, and Thotakura et al. (1987) Meth. Enzymol., 138:350. Further means for producing nonfucosylated antibodies can include the use of cell lines, such as GDP-keto-6-deoxymannose 3,5-epimerase / 4-reductase (FX) knockout CHO cells, which produce antibodies with nonfucosylated N-glycans. Other cell lines that can be used include YB2 / 0 cells, which have reduced levels of Fut8 mRNA. Further means for producing nonfucosylated antibodies are described in Pereira NA, Chan KF, Lin PC, Song Z. The "less-is-more" in therapeutic antibodies: Afucosylated anti-cancer antibodies with enhanced antibody-dependent cellular cytotoxicity. MAbs. 2018 Jul;10(5):693-711, which is incorporated herein by reference in its entirety.

[0036] In embodiments, the anti-ILT7 antibody or antigen-binding fragment can be tagged. In embodiments, the anti-ILT7 antibody or antigen-binding fragment can be tagged with a functional agent. In embodiments, the functional agent is an imaging agent (e.g., a radionuclide, biotin, a fluorescent molecule, a luminescent molecule, or a non-radioactive paramagnetic metal ion). In embodiments, the functional agent is an enzyme (e.g., horseradish peroxidase, alkaline phosphatase, β-galactosidase, or acetylcholinesterase). In embodiments, the functional agent is a drug (e.g., a cytotoxic agent or a radioactive compound).

[0037] dose In embodiments, any of the compositions of the present disclosure comprising an anti-ILT7 antibody or antigen-binding fragment thereof can be administered in any form. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered intravenously, subcutaneously, orally, intramuscularly, intrathecally, sublingually, rectally, vaginally, cutaneously, systemically, topically, transdermally, or by inhalation. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered subcutaneously.

[0038] The anti-ILT7 antibodies or antigen-binding fragments thereof of the present disclosure can be administered at any dose. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at a dose of about: 50 mg, 75 mg, 100 mg, 125 mg, 150 mg, 175 mg, 200 mg, 225 mg, 250 mg, 275 mg, 300 mg, 325 mg, 350 mg, 375 mg, 400 mg, 425 mg, 450 mg, 475 mg, 500 mg, 525 mg, 550 mg, 575 mg, 600 mg, 650 mg, 700 mg, 750 mg, 800 mg, 850 mg, 900 mg, 950 mg, 1000 mg, 1050 mg, 1100 mg, 1150 mg, 1200 mg, 1250 mg, 1300 mg, 1350 mg, 1400 mg, 1450 mg, or about 1500 mg. Any of the above doses can be an effective dose for methods that include prevention, reduction, or elimination.

[0039] In some embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at a dose of approximately 50-100 mg, 50-150 mg, 100-200 mg, 130-180 mg, 150-250 mg, 200-300 mg, 250-350 mg, 50-1500 mg, 75-1200 mg, 100-800 mg, or 150-500 mg. In some embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at a dose selected from the group consisting of 150 mg, 175 mg, 200 mg, 225 mg, 250 mg, 275 mg, 300 mg, 325 mg, 350 mg, 375 mg, 400 mg, 425 mg, 450 mg, 500 mg, 525 mg, 550 mg, 575 mg, and 600 mg. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at an effective dose of about 150 mg. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at an effective dose of about 200 mg. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at an effective dose of about 300 mg.

[0040] Dosage frequency In embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof disclosed herein are administered on a schedule that provides optimal results. In embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof disclosed herein are administered to a subject in need of treatment about once per week, about twice per week, about every two weeks, about every four weeks, about every month, about every six weeks, about every two months, about every three months, about every 12 weeks, about every 15 weeks, about every 16 weeks, about every four months, about every five months, about every six months, or every six months.

[0041] Any number of administrations may be provided to a subject in need thereof. In embodiments, a subject is administered an effective dose daily, weekly, biweekly, or for up to about the lifespan of the subject. In embodiments, administration is given for up to about 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 3 months, 4 months, 5 months, 6 months, 7 months, 8 months, 9 months, 10 months, 11 months, 1 year, 2 years, 3 years, 4 years, or 5 years. In embodiments, a subject receives an effective dose on days 1, 28, 56, 84, 112, 140, 168, 196, 224, 252, 280, and 308 after initiation of treatment. In embodiments, the subject receives an effective dose at weeks 0, 4, 8, 12, 16, 20, 24, 28, 32, 36, 40, and 44 after initiation of treatment. In embodiments, the subject receives an effective dose of 300 mg of an anti-ILT7 antibody or antigen-binding fragment thereof at days 1, 28, 56, 84, 112, 140, 168, 196, 224, 252, 280, and 308 after initiation of treatment. In embodiments, a subject in need of treatment is administered an effective dose of 300 mg of an anti-ILT7 antibody or antigen-binding fragment thereof at weeks 0, 4, 8, 12, 16, 20, 24, 28, 32, 36, 40, and 44 after initiation of treatment. In embodiments, a subject in need of treatment is administered an effective dose of 300 mg of an anti-ILT7 antibody or antigen-binding fragment thereof on day 1, and then every four weeks thereafter, as needed. In embodiments, a subject in need of treatment is administered 300 mg of an anti-ILT7 antibody or antigen-binding fragment thereof every four weeks. Any of the foregoing administrations can vary by about 0 to 7 days, 0 to 5 days, 0 to 4 days, 0 to 3 days, 0 to 2 days, or about 3 days.

[0042] In embodiments, subjects receive an effective dose of an anti-ILT7 antibody or antigen-binding fragment thereof on days 1, 28 (± 3 days), 56 (± 3 days), 84 (± 3 days), 112 (± 3 days), 140 (± 3 days), 168 (± 3 days), 196 (± 3 days), 224 (± 3 days), 252 (± 3 days), 280 (± 3 days), and 308 (± 3 days) after initiation of treatment.

[0043] In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered in an effective single dose. In embodiments, the subject in need of treatment receives at least two doses of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered as a series of multiple doses. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered in an effective single dose or multiple doses.

[0044] In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at a dose of about 300 mg in at least one administration, followed by two doses about once a month or every four weeks. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered as two doses of about 150 mg in at least two administrations, followed by two doses about once a month. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at a dose of about 300 mg about once every four weeks, about once a month, about once every two months, or about once every three months. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at two doses of about 150 mg about every four weeks, about once a month, twice every two months, or twice every three months.

[0045] In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered in two or more doses. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof of the present disclosure is administered to a subject in need of treatment until myositis is prevented, alleviated, or eliminated as measured by a test of the present disclosure. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered as an effective dose of about 300 mg. In embodiments, a method disclosed herein comprises administering an antibody or antigen-binding fragment thereof that binds ILT7 to a subject in need of treatment, wherein the antibody or antigen-binding fragment thereof is administered subcutaneously at an effective dose of about 300 mg. In embodiments, a method disclosed herein comprises administering an antibody or antigen-binding fragment thereof that binds ILT7 to a subject in need of treatment, wherein the antibody or antigen-binding fragment thereof is administered subcutaneously in two doses of about 150 mg each. In aspects, methods disclosed herein comprise administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds ILT7, wherein the antibody or antigen-binding fragment thereof is administered subcutaneously in two doses of about 150 mg each for four weeks. In aspects, methods disclosed herein comprise administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds ILT7, wherein the antibody or antigen-binding fragment thereof is administered subcutaneously in two doses of about 150 mg each for four weeks in at least 12 doses. In aspects, methods disclosed herein comprise administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds ILT7, wherein the antibody or antigen-binding fragment thereof comprises HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively.In aspects, the methods disclosed herein comprise administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds to ILT7, wherein the antibody or antigen-binding fragment thereof comprises HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and wherein the antibody or antigen-binding fragment thereof is administered subcutaneously as a single dose of 300 mg or as two doses of about 150 mg each. In aspects, the methods disclosed herein comprise administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds to ILT7, wherein the antibody or antigen-binding fragment thereof comprises HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and wherein the antibody or antigen-binding fragment thereof is administered subcutaneously as a single dose of 300 mg or as two doses of about 150 mg each for four weeks. In aspects, the methods disclosed herein comprise administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds to ILT7, wherein the antibody or antigen-binding fragment thereof comprises HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and wherein the antibody or antigen-binding fragment thereof is administered subcutaneously as a single dose of 300 mg or as two doses of about 150 mg each over a period of four weeks for at least 12 doses.

[0046] In an aspect, a method disclosed herein comprises administering to a subject in need of treatment an antibody or antigen binding fragment thereof that binds ILT7, in any of the aspects or methods disclosed herein, wherein the antibody or antigen binding fragment thereof comprises a VH and a VL, wherein the VH and VL comprise amino acid sequences having at least about 80%, 85%, 90%, 95%, 99%, or 100% identity to SEQ ID NOs: 2 and 7, respectively. In an aspect, a method disclosed herein comprises administering to a subject in need of treatment an antibody or antigen binding fragment thereof that binds ILT7, wherein the antibody or antigen binding fragment thereof comprises a VH and a VL, wherein the VH and VL comprise the amino acid sequences of SEQ ID NOs: 2 and 7, respectively, wherein the antibody or antigen binding fragment thereof is administered as a single dose of about 300 mg or as two doses of about 150 mg each. In an aspect, a method disclosed herein comprises administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds to ILT7, wherein the antibody or antigen-binding fragment thereof comprises a VH and a VL, wherein the VH and VL comprise the amino acid sequences of SEQ ID NOs: 2 and 7, respectively, and wherein the antibody or antigen-binding fragment thereof is administered subcutaneously as a single dose of about 300 mg or as two doses of about 150 mg each. In an aspect, a method disclosed herein comprises administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds to ILT7, wherein the antibody or antigen-binding fragment thereof comprises a VH and a VL, wherein the VH and VL comprise the amino acid sequences of SEQ ID NOs: 2 and 7, respectively, and wherein the antibody or antigen-binding fragment thereof is administered subcutaneously as a dose of about 300 mg every four weeks. In an aspect, the methods disclosed herein comprise administering to a subject in need of treatment an antibody or antigen-binding fragment thereof that binds to ILT7, wherein the antibody or antigen-binding fragment thereof comprises a VH and a VL, wherein the VH and VL comprise the amino acid sequences of SEQ ID NOs: 2 and 7, respectively, and wherein the antibody or antigen-binding fragment thereof is administered subcutaneously at a dose of about 300 mg every four weeks.

[0047] In embodiments, a subject in need thereof is administered an effective dose of an anti-ILT7 antibody or antigen-binding fragment thereof about once every 2-4 weeks. In embodiments, a subject in need thereof is administered an effective dose of an anti-ILT7 antibody or antigen-binding fragment thereof about once every 4 weeks. In embodiments, a subject in need thereof is administered 300 mg of an anti-ILT7 antibody or antigen-binding fragment thereof for at least three doses every 4 weeks, at least four doses every 4 weeks, at least five doses every 4 weeks, or a combination thereof. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at an effective dose of about 300 mg every 4 weeks. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at an effective dose of about 300 mg monthly. In embodiments, a subject in need of treatment is administered two 150 mg doses of the anti-ILT7 antibody or antigen-binding fragment thereof, once every four weeks for at least three doses, once every four weeks for at least four doses, once every four weeks for at least five doses, or a combination thereof. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered as two 150 mg doses, once every four weeks or monthly.

[0048] method In aspects herein, methods are directed to treating, alleviating, or eliminating myositis diseases or disorders. In aspects, methods include administering an anti-ILT7 antibody or antigen-binding fragment thereof of the present disclosure. In aspects, the anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, alleviate, or eliminate myositis in a subject in need thereof using any of the dosing schedules disclosed herein. Idiopathic inflammatory myopathies (IIM), such as myositis, can be classified into several subgroups: dermatomyositis (including myopathic dermatomyositis), anti-synthetase inflammatory myositis (ASIM), immune-mediated necrotizing myopathy, inclusion body myositis, polymyositis, and overlap myositis. In aspects, a myositis disease or disorder includes dermatomyositis (DM). In aspects, a myositis disease or disorder includes anti-synthetase inflammatory myositis (ASIM). In an embodiment, the myositis disease or disorder comprises polymyositis (PM). In an embodiment, the myositis disease or disorder comprises disseminated inclusion body myositis (sIBM).

[0049] In embodiments, the subject with DM has DM with definite or probable myositis. In embodiments, the subject with DM has a DM rash. In embodiments, the subject with ASIM has ASIM with definite or probable myositis. In embodiments, the subject with ASIM has positive ASIM-related antibodies. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate DM in a subject in need thereof using any of the dosing schedules disclosed herein. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate ASIM in a subject in need thereof using any of the dosing schedules disclosed herein. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate disease in a subject who tests positive for anti-histidyl-tRNA synthetase (e.g., anti-Jo-1) antibodies prior to treatment. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate disease in a subject who has an ASIM diagnosis and tests positive for anti-histidyl-tRNA synthetase (e.g., anti-Jo-1) antibodies prior to treatment. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate disease in a subject who has an ASIM diagnosis and tests positive for ASIM-associated antibodies. In aspects, the anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate disease in a subject whose medical history is positive for one or more of anti-alanyl (e.g., anti-PL-12) antibodies, anti-threonyl (e.g., anti-PL-7) antibodies, anti-asparagyl (e.g., anti-KS) antibodies, anti-glycyl (e.g., anti-EJ) antibodies, anti-isoleucyl (e.g., anti-OJ) antibodies, anti-phenylalanyl-tRNA synthetase (e.g., anti-ZO) antibodies, or anti-tyrosyl (e.g., anti-YRS(HA)) antibodies.In aspects, the anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate disease in a subject with an ASIM diagnosis and whose medical history is positive for one or more of anti-alanyl (e.g., anti-PL-12) antibodies, anti-threonyl (e.g., anti-PL-7) antibodies, anti-asparagyl (e.g., anti-KS) antibodies, anti-glycyl (e.g., anti-EJ) antibodies, anti-isoleucyl (e.g., anti-OJ) antibodies, anti-phenylalanyl-tRNA synthetase (e.g., anti-ZO) antibodies, or anti-tyrosyl (e.g., anti-YRS(HA)) antibodies.

[0050] In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate disease in a subject, and the subject has not been diagnosed with the disease. In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof is administered to prevent, reduce, or eliminate disease in a subject, and the subject has not been diagnosed with immune-mediated necrotizing myopathy (IMNM), inclusion body myositis (IBM), or drug-induced myositis. In embodiments, a subject is diagnosed with IMNM if the subject has 3-hydroxy-3-methylglutaryl-coenzyme A reductase (i.e., anti-HMGR) antibodies, anti-signal recognition particle (i.e., anti-SRP) antibodies, or is autoantibody negative. In embodiments, a subject is diagnosed with IBM if the subject has anti-cytosolic 5'-nucleotidase 1A (i.e., anti-CN1A) antibodies.

[0051] In embodiments, a method of treatment in a subject in need thereof comprises administering an effective amount of an anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the administration is effective in abating a disease or disorder as compared to a) a disease or disorder in an otherwise comparable subject lacking administration; or b) a baseline measurement of the disease or disorder in need of treatment. In embodiments, the administration is effective in reducing the level of type I interferon (IFN, e.g., IFN-α) in the subject in need of treatment as compared to baseline levels in the subject. In embodiments, the level of type I IFN is reduced by at least about, or by up to about, 1-fold, 5-fold, 10-fold, 25-fold, 50-fold, 75-fold, 100-fold, 150-fold, or 250-fold in a subject administered the anti-ILT7 antibody or antigen-binding fragment as compared to baseline levels in the subject.

[0052] In embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof are used to treat a myositis disease or disorder. In embodiments, the anti-ILT7 antibodies or antigen-binding fragments thereof are used to treat a subject in need thereof using any of the dosing schedules disclosed herein.

[0053] In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at a dose of about 300 mg. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered two times at a dose of about 150 mg. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered subcutaneously at a dose of about 300 mg. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered subcutaneously two times at a dose of about 150 mg. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at a dose of about 300 mg every four weeks. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered two times at a dose of about 150 mg every four weeks. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered in at least two doses of about 150 mg every four weeks. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered at a dose of about 150 mg for two doses every four weeks for at least three doses. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered subcutaneously at a dose of about 300 mg for at least two doses every four weeks. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered subcutaneously at a dose of about 300 mg for at least three doses every four weeks.

[0054] In embodiments, compositions comprising an anti-ILT7 antibody or antigen-binding fragment thereof are formulated for administration. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is at least about, or at most about, 1 mg / mL, 2 mg / mL, 3 mg / mL, 4 mg / mL, 5 mg / mL, 6 mg / mL, 7 mg / mL, 8 mg / mL, 9 mg / mL, 10 mg / mL, 15 mg / mL, 20 mg / mL, 25 mg / mL, 30 mg / mL, 35 mg / mL, 40 mg / mL, 45 mg / mL, 50 mg / mL, 55 mg / mL, 60 mg / mL, 65 mg / mL, 70 mg / mL, L, 75mg / mL, 80mg / mL, 85mg / mL, 90mg / mL, 95mg / mL, 100mg / mL, 105mg / mL, 110mg / mL, 115mg / mL, 120mg / mL, 125mg / mL, 130mg / mL, 135mg / mL, 140mg / mL, 145mg / mL, 150mg / mL, 155mg / mL, 160mg / mL, 165mg / mL, 170mg / mL, 175mg / mL, 180mg / mL, 185mg / m L, 190mg / mL, 195mg / mL, 200mg / mL, 205mg / mL, 210mg / mL, 215mg / mL, 220mg / mL, 225mg / mL, 230mg / mL, 235mg / mL, 240mg / mL, 245mg / mL, 250mg / mL, 255mg / mL, 260mg / mL, 265mg / mL, 270mg / mL, 275mg / mL, 280mg / mL, 285mg / mL, 290mg / mL, 295mg / mL, 30 The anti-ILT7 antibody or antigen-binding fragment thereof may be formulated at a concentration of about 100 mg / mL. An effective dose of an embodiment anti-ILT7 antibody or antigen-binding fragment thereof may be formulated at a concentration of about 150 mg / mL.

[0055] The doses and administration regimens of the anti-ILT7 antibodies or antigen-binding fragments thereof disclosed herein for treating any myositis disease or disorder can be such that any therapeutic effect achieved from administration of the anti-ILT7 antibody or antigen-binding fragment thereof can be considered "long-lasting." A "long-lasting" effect of an anti-ILT7 antibody or antigen-binding fragment thereof in treating a myositis disease or disorder is one in which the therapeutic effect achieved by the anti-ILT7 antibody or antigen-binding fragment thereof is maintained for at least 4 weeks, at least 6 weeks, at least 8 weeks, at least 10 weeks, at least 12 weeks, at least 16 weeks, at least 20 weeks, at least 24, at least 28 weeks, at least 32 weeks, at least 36 weeks, at least 40 weeks, at least 44 weeks, at least 48 weeks, at least 1 year, at least 2 years, or up to about at least 5 years after the previous administration of the anti-ILT7 antibody or antigen-binding fragment thereof (even though the anti-ILT7 antibody or antigen-binding fragment thereof is no longer administered).

[0056] In aspects, administration of an anti-ILT7 antibody or antigen-binding fragment thereof is effective to eliminate disease in a subject in need thereof. In aspects, administration of an anti-ILT7 antibody or antigen-binding fragment thereof is effective to alleviate disease in a subject in need thereof. In some cases, administration of an anti-ILT7 antibody or antigen-binding fragment thereof is effective to eliminate or alleviate disease in a subject in need thereof for at least about 1 week, 2 weeks, 3 weeks, 1 month, 2 months, 3 months, 4 months, 5 months, 6 months, 7 months, 8 months, 9 months, 10 months, 11 months, 1 year, 2 years, 3 years, 4 years, at least about 5 years, or for the lifetime of the subject. In aspects, administration of an anti-ILT7 antibody or antigen-binding fragment thereof is effective to eliminate disease in a subject in need thereof for at least about 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, or at least about 12 weeks. In aspects, administration of an anti-ILT7 antibody or antigen-binding fragment thereof is effective to eliminate or reduce disease for at least about 1 month, 2 months, 3 months, 4 months, 5 months, 6 months, 7 months, 8 months, 9 months, 10 months, 11 months, or at least about 12 months in a subject in need thereof. In aspects, administration of an anti-ILT7 antibody or antigen-binding fragment thereof is effective to eliminate or reduce disease for at least about 1 year, 2 years, 3 years, 4 years, 5 years, 6 years, 7 years, 8 years, 9 years, or at least about 10 years in a subject in need thereof. In aspects, administration of an anti-ILT7 antibody or antigen-binding fragment thereof is effective to eliminate or reduce disease for the lifetime of a subject in need thereof.

[0057] In embodiments, the subject in need of treatment has been administered one or more standard of care therapies for treating a myositis disease or disorder prior to administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the standard of care therapies include corticosteroids, disease-modifying antirheumatic drugs (DMARDs), and / or immunomodulators. In embodiments, the subject continues the standard of care therapy while the anti-ILT7 antibody or antigen-binding fragment thereof is administered. In embodiments, the standard of care therapy is administered as a second therapy. In embodiments, any of the referenced therapies can be combined in a therapeutic regimen for the treatment of a myositis disease or disorder. In embodiments, the subject is treated with both the anti-ILT7 antibody or antigen-binding fragment thereof and the standard of care therapy.

[0058] In embodiments, any standard of care therapy is combined as part of a treatment regimen or combination therapy. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof is administered sequentially with any therapy disclosed herein. In embodiments, the anti-ILT7 antibody or antigen-binding fragment thereof can generally be administered simultaneously with any second therapy disclosed herein. In embodiments, the subject in need of treatment has been administered any standard of care therapy for treating a myositis disease or disorder prior to administration of the anti-ILT7 antibody or antigen-binding fragment thereof.

[0059] In embodiments, an anti-ILT7 antibody or antigen-binding fragment thereof is administered to a subject in need of treatment. In embodiments, the subject in need of treatment has a myositis disease or disorder. In embodiments, the subject has a myositis disease or disorder, and the disease is dermatomyositis (DM). In embodiments, the subject has a myositis disease, and the disease is anti-synthetase inflammatory myositis (ASIM). In embodiments, the subject in need of treatment is treated with a method comprising co-administration of an immunomodulatory agent and an anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the subject in need of treatment is treated with a method comprising co-administration of a DMARD and an anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the DMARD and the immunomodulatory agent are the same agent. In embodiments, the subject in need of treatment is treated with a method comprising co-administration of a corticosteroid and an anti-ILT7 antibody or antigen-binding fragment thereof.

[0060] Methods of the present disclosure can include tapering one or more standard of care treatments and / or an anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the method comprises tapering a standard of care therapy. In embodiments, the method comprises tapering a corticosteroid. In embodiments, the method comprises tapering prednisone. Tapering can occur at any time. In embodiments, tapering occurs prior to administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, tapering occurs simultaneously with administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, tapering occurs after administration of the anti-ILT7 antibody or antigen-binding fragment thereof.

[0061] DMARD In embodiments, the subject is administered a DMARD. In embodiments, the subject is administered a DMARD in combination with an anti-ILT7 antibody or antigen-binding fragment thereof disclosed in any of the methods herein. In embodiments, the DMARD is a conventional DMARD (cDMARD). In embodiments, the DMARD is a biologic DMARD (bDMARD). Exemplary DMARDs are selected from the group consisting of, but not limited to, methotrexate, sulfasalazine, leflunomide, hydroxychloroquine, plaquenil, cyclosporine, etanercept, baricitinib, tofacitinib, upadacitinib, infliximab, adalimumab, certolizumab, and golimumab, and combinations thereof. In embodiments, the DMARD is plaquenil or cyclosporine.

[0062] immunomodulators In embodiments, the subject is administered an immunomodulatory agent. In embodiments, the subject is administered an immunomodulatory agent in combination with an anti-ILT7 antibody or antigen-binding fragment thereof disclosed in any of the methods herein. In embodiments, the immunomodulatory agent comprises a cell modifying agent, an interleukin, or a pathway modifier. In embodiments, the immunomodulatory agent is selected from the group consisting of, but not limited to, rituximab, ofatumumab, obinutuzumab, ibritumomab, azathioprine, abatacept, belatacept, adalimumab, cyclophosphamide, mycophenolate mofetil, thymoglobulin, alemtuzumab, basiliximab, cyclosporine, sirolimus, tacrolimus, everolimus, etc. In embodiments, the immunomodulatory agent is azathioprine, cyclosporine, mycophenolate mofetil, or tacrolimus.

[0063] Corticosteroids In embodiments, the subject is administered a corticosteroid. In embodiments, the subject is administered a corticosteroid in combination with an anti-ILT7 antibody or antigen-binding fragment thereof disclosed in any of the methods herein. In embodiments, the corticosteroid comprises a glucocorticoid. Glucocorticoids (GCs) are steroid hormones for use in treating inflammatory, myositis, and / or cancer diseases or disorders. GCs bind to the GC receptor (GR), which belongs to the nuclear receptor superfamily of transcription factors, to exert a wide range of physiological and therapeutic effects. In embodiments, one or more GCs are administered to a subject in need of treatment. In embodiments, the one or more GCs are selected from the group consisting of, but not limited to, triamcinolone, methylprednisolone, budesonide, dexamethasone, triamcinolone, prednisone, hydrocortisone, dexamethasone, betamethasone, prednisolone, deflazacort, and combinations thereof. In embodiments, the GC is prednisone. In an embodiment, the GC is methylprednisolone.

[0064] In aspects, treatment with standard of care therapy in combination with an anti-ILT7 antibody or antigen-binding fragment thereof is more effective at controlling disease activity than maintaining standard of care therapy alone or substituting an anti-ILT7 antibody or antigen-binding fragment thereof for a partially effective standard of care therapy. In aspects, treatment with standard of care therapy in combination with an anti-ILT7 antibody or antigen-binding fragment thereof is at least about 1-fold, 10-fold, 50-fold, 100-fold, 200-fold, 500-fold, or up to 1000-fold effective in reducing disease or symptoms in a subject in need thereof compared to an otherwise comparable subject who is not administered the anti-ILT7 antibody or antigen-binding fragment thereof. In aspects, treatment with standard of care therapy in combination with an anti-ILT7 antibody or antigen-binding fragment thereof is more effective at stabilizing disease than maintaining standard of care therapy alone or substituting an anti-ILT7 antibody or antigen-binding fragment thereof for a partially effective standard of care therapy.

[0065] In embodiments, the one or more standard of care therapies are administered about 1, about 2, about 3, about 4, about 5, about 6, about 7, about 8, about 9, about 10, about 11, about 12, about 13, about 14, about 15, about 20, about 25, about 30, about 35, about 40, about 52, about 52 or more weeks prior to administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the one or more standard of care therapies are administered about 4 weeks prior to administration of the anti-ILT7 antibody or antigen-binding fragment thereof.

[0066] The doses and administration regimens of the anti-ILT7 antibodies or antigen-binding fragments thereof disclosed herein for treating any myositis disease or disorder can be such that any therapeutic effect achieved from administration of the anti-ILT7 antibody or antigen-binding fragment thereof can be considered "long-lasting." A "long-lasting" effect of an anti-ILT7 antibody or antigen-binding fragment thereof in treating a myositis disease or disorder is one in which the therapeutic effect achieved by the anti-ILT7 antibody or antigen-binding fragment thereof is maintained for at least 4 weeks, at least 6 weeks, at least 8 weeks, at least 10 weeks, at least 12 weeks, at least 16 weeks, at least 20 weeks, at least 24 weeks, at least 28 weeks, at least 32 weeks, at least 36 weeks, at least 40 weeks, at least 44 weeks, at least 48 weeks, or at least 52 weeks after administration of the final dose of the anti-ILT7 antibody or antigen-binding fragment thereof in a series (while no more anti-ILT7 antibody or antigen-binding fragment thereof is administered). In embodiments, it may be advantageous to reduce the frequency of administration of any of the compositions provided herein. Exemplary advantages of less frequent administration include, but are not limited to, a reduced frequency of side effects associated with the administered composition, a reduced toxicity associated with treatment, an increased quality of life for the treated subject, and the like.

[0067] investigation In embodiments, a subject is monitored. In embodiments, a subject is monitored as part of a treatment. In embodiments, a subject with a confirmed or suspected myositis disease or disorder is monitored as part of a treatment. Monitoring can occur at any time before, during, or after administration of an anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, monitoring occurs before administration begins. In embodiments, monitoring occurs simultaneously with administration. In embodiments, monitoring occurs after administration has ended.

[0068] In embodiments, subjects are surveyed and baseline disease measures are determined. In embodiments, subjects are surveyed and baseline disease measures are determined at any time before, during, or after administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the survey is conducted and baseline disease measures are determined prior to administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the survey is conducted and baseline disease measures are determined concurrently with administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the survey is conducted and baseline disease measures are determined after administration of the anti-ILT7 antibody or antigen-binding fragment thereof.

[0069] In embodiments, baseline disease measures increase or decrease in response to administration, or lack thereof, of the present disclosure. In embodiments, baseline disease measures may increase, which may indicate effective treatment of the disease or disorder. In embodiments, baseline disease measures may decrease, which may indicate effective treatment of the disease or disorder. In embodiments, baseline disease measures increase or decrease after administration of an anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, baseline disease measures may increase after administration of an anti-ILT7 antibody or antigen-binding fragment, which may indicate effective treatment of the disease or disorder. In embodiments, baseline disease measures may decrease after administration of an anti-ILT7 antibody or antigen-binding fragment, which may indicate effective treatment of the disease or disorder.

[0070] In embodiments, the baseline disease measure is compared to the disease measure in the subject after administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the disease measure in the subject administered the anti-ILT7 antibody or antigen-binding fragment thereof is increased compared to the baseline disease measure measured before administration of the anti-ILT7 antibody or antigen-binding fragment. In embodiments, the disease measure in the subject administered the anti-ILT7 antibody or antigen-binding fragment thereof is decreased compared to the baseline disease measure measured before administration of the anti-ILT7 antibody or antigen-binding fragment. In embodiments, the disease measure in the subject administered the anti-ILT7 antibody or antigen-binding fragment thereof is increased compared to the baseline disease measure measured before administration of the anti-ILT7 antibody or antigen-binding fragment, indicating effective treatment. In embodiments, the disease measure in the subject administered the anti-ILT7 antibody or antigen-binding fragment thereof is decreased compared to the baseline disease measure measured before administration of the anti-ILT7 antibody or antigen-binding fragment, indicating effective treatment.

[0071] In embodiments, the investigation includes measuring the efficacy of the treatment. Efficacy can be measured by any of the investigations disclosed herein. In embodiments, efficacy in preventing, alleviating, or eliminating a myositis disease or disorder (e.g., DM or ASIM) is measured by detecting a baseline change in the level of pDCs in the subject. In embodiments, efficacy in preventing, alleviating, or eliminating a myositis disease or disorder is measured by assessing pharmacokinetic parameters of an anti-ILT7 antibody or antigen-binding fragment thereof used to treat a subject in need of treatment, a baseline change in the blood concentration of pDCs in the subject, the presence of anti-drug antibodies (ADAs), and combinations thereof. In embodiments, efficacy in preventing, alleviating, or eliminating a myositis disease or disorder is measured by measuring pharmacokinetic parameters of an anti-ILT7 antibody or antigen-binding fragment thereof used to treat a subject in need of treatment. In embodiments, efficacy in preventing, alleviating, or eliminating a myositis disease or disorder is measured by measuring a baseline change in the level of pDCs in the subject. In embodiments, efficacy in preventing, alleviating, or eliminating a myositis disease or disorder is measured by measuring the presence of ADAs. In an embodiment, the myositis disease or disorder includes DM or ASIM.

[0072] Any of the surveys referenced below can be conducted at any time. In embodiments, subjects are surveyed every minute, hourly, daily, weekly, monthly, quarterly, or yearly. In embodiments, surveys are completed twice daily, every other week, every other month, or every six months.

[0073] In some embodiments, the study is conducted on days -28, -27, -26, -25, -24, -23, -22, -21, -20, -19, -18, -17, -16, -15, -14, -13, -12, -11, -10, -9, -8, -7, -6, -5, -4, -3, -2, -1, from day 0, 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 81, 82, 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, 97, 98, 99, 100, Day, 18th, 19th, 20th, 21st, 22nd, 23rd, 24th, 25th, 26th, 27th, 28th, 29th, 30th, 31st, 32nd, 33rd, 34th, 35th, 36th, 37th, 38th, 39th, 40th, 41st, 42nd, 43rd, 44th, 45th, 46th, 47th, 48th, 49th, 50th, 51st, 52nd, 53rd, 54th, 55th, 56th, 57th, 58th, 59th, 60th, 61st, 62nd, 63rd, 64th, 65th, 66th, 67th Day, 68th, 69th, 70th, 71st, 72nd, 73rd, 74th, 75th, 76th, 77th, 78th, 79th, 80th, 81st, 82nd, 83rd, 84th, 85th, 86th, 87th, 88th, 89th, 90th, 91st, 92nd, 93rd, 94th, 95th, 96th, 97th, 98th, 99th, 100th, 101st, 102nd, 103rd, 104th, 105th, 106th, 107th, 108th, 109th, 110th, 111th, 112th, 113th, 114th Day 4, Day 115, Day 116, Day 117, Day 118, Day 119, Day 120, Day 121, Day 122, Day 123, Day 124, Day 125, Day 126, Day 127, Day 128, Day 129, Day 130, Day 131, Day 132, Day 133, Day 134, Day 135, Day 136, Day 137, Day 138, Day 139, Day 140, Day 141, Day 142, Day 143, Day 144, Day 145, Day 146, Day 147, Day 148, Day 149, Day 150, Day 151, Day 152, Day 153, Day 154, Day 155,Day 156, Day 157, Day 158, Day 159, Day 160, Day 161, Day 162, Day 163, Day 164, Day 165, Day 166, Day 167, Day 168, Day 169, Day 170, Day 171, Day 172, Day 173, Day 174, Day 175, Day 176, Day 177, Day 178, Day 179, Day 180, Day 181, Day 182, Day 183, Day 184, Day 185, Day 186, Day 187, Day 188, Day 189, Day 190, Day 191, Day 192, Day 193, Day 194, Day 195, Day 196, Day 197 Day, 198th day, 199th day, 200th day, 201st day, 202nd day, 203rd day, 204th day, 205th day, 206th day, 207th day, 208th day, 209th day, 210th day, 211th day, 212th day, 213th day, 214th day, 215th day, 216th day, 217th day, 218th day, 219th day, 220th day, 221st day, 222nd day, 223rd day, 224th day, 225th day, 226th day, 227th day, 228th day, 229th day, 230th day, 231st day, 232nd day, 233rd day, 234th day, 235th day, 236th day, 237th day, 238th day, Day 239, Day 240, Day 241, Day 242, Day 243, Day 244, Day 245, Day 246, Day 247, Day 248, Day 249, Day 250, Day 251, Day 252, Day 253, Day 254, Day 255, Day 256, Day 257, Day 258, Day 259, Day 260, Day 261, Day 262, Day 263, Day 264, Day 265, Day 266, Day 267, Day 268, Day 269, Day 270, Day 271, Day 272, Day 273, Day 274, Day 275, Day 276, Day 277, Day 278, Day 279, Day 280 Day, 281st, 282nd, 283rd, 284th, 285th, 286th, 287th, 288th, 289th, 290th, 291st, 292nd, 293rd, 294th, 295th, 296th, 297th, 298th, 299th, 300th, 301st, 302nd, 303rd, 304th, 305th, 306th, 307th, 308th, 309th, 310th, 311th, 312th, 313th, 314th, 315th, 316th, 317th, 318th, 319th, 320th, 321st,Day 322, Day 323, Day 324, Day 325, Day 326, Day 327, Day 328, Day 329, Day 330, Day 331, Day 332, Day 333, Day 334, Day 335, Day 336, Day 337, Day 338, Day 339, Day 340, Day 341, Day 342, Day 343, Day 344, Day 345, Day 346, Day 347, Day 348, Day 349, Day 350, Day 351, Day 352, Day 353, Day 354, Day 355, Day 356, Day 357, Day 358, Day 359, Day 360, Day 361, The surveys are conducted on days 362, 363, 364, 365, 366, 367, 368, 369, 370, 371, 372, 373, 374, 375, 376, 377, 378, 379, 380, 381, 382, ​​383, 384, 385, 386, 387, 388, 389, 390, 391, 392, 393, 394, 395, 396, 397, 398, or up to about day 399. The timing of the surveys is further described below.

[0074] Inclusion research In embodiments, a subject in need thereof is evaluated prior to initiating treatment. Exemplary investigation or inclusion criteria include subjects with one or more chronic (i.e., stable) myositis diseases or disorders.

[0075] In embodiments, the subject in need of treatment can be either male or female. In embodiments, the subject in need of treatment is an adult subject. In embodiments, the subject in need of treatment can be between about 18 and 75 years of age.

[0076] In embodiments, the subject in need of treatment has a definite or probable diagnosis of myositis. In embodiments, the subject in need of treatment has a definite or probable diagnosis of myositis according to the American College of Rheumatology (ACR) criteria. In embodiments, the subject in need of treatment has a definite or probable diagnosis of myositis according to the European League Against Rheumatism (EULAR) criteria. In embodiments, the subject in need of treatment has a definite or probable diagnosis of myositis according to the European League Against Rheumatism (EULAR) 2017 criteria. In embodiments, the ACR or EULAR 2017 total summary score excluding muscle biopsy is 5.5 or greater. In embodiments, the ACR or EULAR 2017 total summary score including muscle biopsy is 6.7 or greater. In embodiments, the subject in need of treatment has a pathologic skin rash (e.g., heliotrope rash, Gottron papules, and / or Gottron sign) that is not assessed by the ACR or EULAR 2017 criteria.

[0077] Idiopathic inflammatory myopathies (IIM), such as myositis, can be divided into several subgroups: dermatomyositis (including myopathic dermatomyositis), antisynthetase inflammatory myositis (ASIM), immune-mediated necrotizing myopathy, inclusion body myositis, polymyositis, and overlap myositis. In certain embodiments, "polymyositis" includes ASIM and other forms of inflammatory myositis. In embodiments, a subject in need of treatment may have a probable or definite myositis diagnosis, including, but not limited to, dermatomyositis (DM), antisynthetase inflammatory myositis (ASIM), polymyositis, or both DM and ASIM, or both DM and polymyositis.

[0078] In embodiments, the subject in need of treatment has a diagnosis of DM. In embodiments, the subject in need of treatment has a diagnosis of DM with a current DM rash prior to initiating treatment. In embodiments, the subject in need of treatment has a diagnosis of DM with a history of DM rash prior to initiating treatment. In embodiments, the subject in need of treatment has a diagnosis of ASIM. In embodiments, the subject in need of treatment has a diagnosis of ASIM prior to initiating treatment and tests positive for anti-Jo-1 antibodies. In embodiments, the subject in need of treatment has a diagnosis of ASIM and undergoes a medical history test prior to initiating treatment that tests positive for anti-PL-12, anti-PL-7, anti-KS, anti-EJ, anti-OJ, anti-ZO, or anti-YRS (HA) antibodies. In embodiments, the subject in need of treatment has a manual muscle testing (MMT8) score of less than 142 on the Myositis Damage Index (MDI), at least two or more Core Set Scales (CSM), and a total muscle damage score of 5 or less on a 10-cm VAS prior to initiating treatment. In embodiments, the two CSMs are selected from the group consisting of PtGDA ≥ 2 cm on a 10 cm visual analog scale (VAS), Physician-Assessed Total Disease Activity (PhGDA) ≥ 2 cm on a 10 cm VAS, extramuscular activity ≥ 2 cm on a 10 cm VAS, at least one muscle enzyme 1.5 times the upper limit of normal (ULN), and Health Assessment Questionnaire Disability Index (HAQ-DI) ≥ 0.5.

[0079] In embodiments, a subject in need of treatment may have prior and / or concomitant therapy for the treatment of a myositis disorder at the time of initiation of treatment. In embodiments, a subject in need of treatment may have stable administration of azathioprine, tacrolimus, cyclosporine, plaquenil, and / or mycophenolate mofetil for at least four weeks prior to initiation of treatment. In embodiments, a subject in need of treatment may have stable administration of methotrexate for at least four weeks prior to initiation of treatment. In embodiments, a subject in need of treatment may have stable administration of a corticosteroid (e.g., prednisone or methylprednisolone) for at least four weeks prior to initiation of treatment.

[0080] immunogenicity In embodiments, the investigation includes measuring the level of immunogenicity, if any, of the anti-ILT7 antibody or antigen-binding fragment thereof of the present disclosure. Immunogenicity includes measuring the presence of anti-drug antibodies (ADA) against the anti-ILT7 antibody or antigen-binding fragment thereof. The presence of ADA can be assessed using plasma samples from subjects to whom the anti-ILT7 antibody or antigen-binding fragment thereof is administered. In embodiments, ADA is not detected after administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, ADA levels are reduced compared to an otherwise comparable method in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof; for example, the reduction can be about 10%, 15%, 20%, 25%, 50%, 60%, 70%, 80%, 90%, 95%, 96%, 97%, 98%, 99%, or up to 100% compared to ADA levels in an otherwise comparable method in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof.

[0081] In embodiments, ADA levels for the anti-ILT7 antibody or antigen-binding fragment thereof are measured at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day 70, day 71, day 72, day The study may be conducted at weeks 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, or at least about 56 weeks. In embodiments, measurements of ADA levels to an anti-ILT7 antibody or antigen-binding fragment thereof can be investigated at about day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 36 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days after initiation of treatment.

[0082] skin swab In embodiments, the investigation can include investigating the skin microbiome. In embodiments, the skin microbiome is sampled using a skin swab. In embodiments, a skin microbiome profile is collected and compared to a baseline level. In embodiments, the skin microbiome profile changes by at least about 0.5-fold, 1-fold, 1.5-fold, 1-fold, 2-fold, 3-fold, 4-fold, 5-fold, 10-fold, 20-fold, 40-fold, 60-fold, 80-fold, 100-fold, 120-fold, 140-fold, 160-fold, 180-fold, 200-fold, 220-fold, 240-fold, 260-fold, 280-fold, or up to about 300-fold after administration compared to the baseline. Suitable methods for investigating the skin microbiome can include qPCR, DNA sequencing, RNA sequencing, and exome sequencing, as well as combinations thereof.

[0083] In embodiments, changes in the skin microbiome profile in response to an anti-ILT7 antibody or antigen-binding fragment thereof are measured at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day 69, day 70 The study may be conducted at day 1, day 6, week 1, week 2, week 3, week 4, week 5, week 6, week 7, week 8, week 9, week 10, week 11, week 12, week 13, week 14, week 15, week 16, week 17, week 18, week 19, week 20, week 21, week 22, week 23, week 24, week 25, week 26, week 27, week 28, week 29, week 30, week 31, week 32, week 33, week 34, week 35, week 36, week 37, week 38, week 39, week 40, week 41, week 42, week 43, week 44, week 45, week 46, week 47, week 48, week 49, week 50, week 51, week 52, week 53, week 54, week 55, or at least about week 56. In embodiments, measurement of changes in the skin microbiome profile in response to the anti-ILT7 antibody or antigen-binding fragment thereof can be examined at about day 1 and at 24 weeks ± 3 days after initiation of treatment.

[0084] Whole blood transcriptomics In embodiments, the investigation involves quantifying expression levels of genes or gene pathways associated with disease activity by whole blood transcriptomics. In embodiments, whole blood transcriptomics can be investigated by isolating RNA from whole blood. In embodiments, RNA testing can be performed to investigate changes in gene expression or gene pathways associated with myositis over time, the mechanism of action of or response to administration of an anti-ILT7 antibody or antigen-binding fragment thereof, or the etiology of myositis.

[0085] RNA can be isolated from whole blood on a regular basis and examined for changes in gene expression levels or gene pathways associated with disease activity. In embodiments, transcriptome profiling is investigated by methods including, but not limited to, qPCR, RNAseq, and exome sequencing.

[0086] In embodiments, the expression level of genes associated with disease activity by RNA analysis can be reduced by at least about 1-fold, 2-fold, 3-fold, 4-fold, 5-fold, 10-fold, 20-fold, 40-fold, 60-fold, 80-fold, 100-fold, 120-fold, 140-fold, 160-fold, 180-fold, 200-fold, 220-fold, 240-fold, 260-fold, 280-fold, or up to about 300-fold after administration, compared to an otherwise comparable method in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the expression level of genes associated with disease activity by RNA analysis can be reduced by at least about 10%, 15%, 20%, 25%, 50%, 60%, 70%, 80%, 90%, 95%, 96%, 97%, 98%, 99%, or up to 100% compared to an otherwise comparable method.

[0087] In embodiments, measurement of gene expression levels for an anti-ILT7 antibody or antigen-binding fragment thereof associated with disease activity by RNA analysis is performed on about day -28, day -27, day -26, day -25, day -24, day -23, day -22, day -21, day -20, day -19, day -18, day -17, day -16, day -15, day -14, day -13, day -12, day -11, day -10, day -9, day -8, day -7, day -6, day -5, day -4, day -3, day -2, day -1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day 70, day The study can be conducted at the following time points: day 1, day 5, day 6, week 1, week 2, week 3, week 4, week 5, week 6, week 7, week 8, week 9, week 10, week 11, week 12, week 13, week 14, week 15, week 16, week 17, week 18, week 19, week 20, week 21, week 22, week 23, week 24, week 25, week 26, week 27, week 28, week 29, week 30, week 31, week 32, week 33, week 34, week 35, week 36, week 37, week 38, week 39, week 40, week 41, week 42, week 43, week 44, week 45, week 46, week 47, week 48, week 49, week 50, week 51, week 52, week 53, week 54, week 55, or at least about week 56. In embodiments, measurement of expression levels of genes for anti-ILT7 antibodies or antigen-binding fragments thereof associated with disease activity by RNA analysis can be investigated at about day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days after initiation of treatment. In embodiments, whole blood samples can be collected and investigated by RNA analysis. In embodiments, whole blood samples can be collected and investigated by metabolomics. In embodiments, whole blood samples can be collected and investigated by proteomics. In embodiments, whole blood samples can be collected and investigated by RNA analysis, metabolomics, and / or proteomics.

[0088] Genetics In embodiments, the investigation includes determining the sequence of genes associated with disease activity by whole blood DNA analysis. In embodiments, DNA testing can be performed to investigate changes in gene sequence over time, including single nucleotide polymorphisms (SNPs) associated with the development of myositis (e.g., DM or ASIM), or Fcγ receptors, in genes that may be associated with the mechanism of action of an anti-ILT7 antibody or antigen-binding fragment thereof.

[0089] In some embodiments, whole blood can be collected and used to assess gene sequences before, during, and after treatment with the compositions provided herein. Gene sequences found to be regulated by treatment can be analyzed in whole blood using quantitative methods. Samples can be used to examine gene sequences and their changes over time, as examined by NGS, Sanger sequencing, or PCR.

[0090] In embodiments, measurement of gene sequence changes associated with disease activity and / or treatment efficacy for an anti-ILT7 antibody or antigen-binding fragment thereof by DNA analysis is performed on about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day The study may be conducted at day 1, day 4, day 5, day 6, 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, determination of gene sequence changes associated with disease activity and / or treatment efficacy for an anti-ILT7 antibody or antigen-binding fragment thereof by DNA analysis can be examined at about day 1 after initiation of treatment.

[0091] Pharmacokinetic survey In embodiments, the methods provided herein can include measuring the concentration of an anti-ILT7 antibody or antigen-binding fragment thereof in a subject in need of treatment after administration. In embodiments, the methods include a pharmacokinetic study. In embodiments, the sample is a blood sample or a plasma sample, or a combination of both. In embodiments, suitable assays for measuring pharmacokinetics can include electrochemiluminescence (ECL) assays, bead-based assays, cell-based assays, and combinations thereof. In embodiments, the sample can include plasma, and the plasma can include a maximum observed concentration (C max ), area under the concentration-time curve (AUC), clearance (CL), and terminal elimination half-life (t 1 / 2 ) to investigate the concentration of the anti-ILT7 antibody or antigen-binding fragment thereof.

[0092] In embodiments, pharmacokinetic studies for an anti-ILT7 antibody or antigen-binding fragment thereof are performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 days, 18 days, 19 days, 20 days, 21 days, 22 days, 23 days, 24 days, 25 days, 26 days, 27 days, 28 days, 29 days, 30 days, 31 days, 32 days, 33 days, 34 days, 35 days, 36 days, 37 days, 38 days, 39 days, 40 days, 41 days, 42 days, 43 days, 44 days, 45 days, 46 days, 47 days, 48 ​​days, 49 days, 50 days, 51 days, 52 days, 53 days, 54 days, 55 days, 56 days, 57 days, 58 days, 59 days, 60 days, 61 days, 62 days, 63 days, 64 days, 65 days, 66 days, 67 days The study can be conducted at 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, pharmacokinetic studies for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about day 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days after initiation of treatment.

[0093] Pharmacodynamics survey In embodiments, the method includes a pharmacodynamic study. In embodiments, the study can be conducted over a period of time. In embodiments, the sample includes a blood sample, a serum sample, a plasma sample, a skin sample, or a muscle sample. In embodiments, the location of the muscle biopsy can be identified by MRI. In embodiments, the muscle biopsy can be taken by non-invasive surgery (e.g., needle biopsy) or invasive surgery (i.e., surgical biopsy). In embodiments, the skin biopsy can be taken by punch biopsy.

[0094] In embodiments, the methods provided herein can include measuring the reduction or elimination of biomarkers, including but not limited to, pDCs, IFN-α, myxovirus resistance protein A (MxA), IFN gene signatures, and peripheral blood mononuclear cells (PBMCs), by the anti-ILT7 antibody or antigen-binding fragment thereof in a subject in need of treatment following administration. In embodiments, the reduction of the biomarkers can be detected relative to an otherwise comparable method in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the elimination of the biomarkers can be detected relative to an otherwise comparable method in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof, relative to an otherwise comparable method except for the lack of administration of the anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the reduction in biomarkers is at least about, or up to about: 1 fold, 2 fold, 3 fold, 4 fold, 5 fold, 10 fold, 15 fold, 20 fold, 25 fold, 30 fold, 35 fold, 40 fold, 45 fold, 50 fold, 55 fold, 60 fold, 65 fold, 70 fold, 75 fold, 80 fold, 85 fold, 90 fold, 95 fold, 100 fold, 105 fold, and up to about 300 fold. In embodiments, the treatment efficacy of an anti-ILT7 antibody or antigen-binding fragment thereof on a biomarker can be investigated over time using a suitable immunoassay. In embodiments, the efficacy of an anti-ILT7 antibody or antigen-binding fragment thereof is investigated over time using a quantified immunoassay.

[0095] The present disclosure provides anti-ILT7 antibody-containing compositions that effectively reduce or deplete pDCs in a subject. Because the anti-ILT7 antibody or antigen-binding fragment thereof binds to and depletes a biomarker, reduction or elimination can be used as a measure of treatment efficacy. In embodiments, total pDCs are a measure of target binding. Suitable assays for investigating pDC levels include flow cytometry, histology, immunohistochemistry, blood analysis, microscopy, PCR, ELISA, and combinations thereof.

[0096] The present disclosure provides anti-ILT7 antibodies or antigen-binding fragments thereof containing compositions that effectively reduce or eliminate blood MxA levels, cytokines, chemokines, or other proteins (e.g., IFN-α, IL-17, and IFN-γ), PBMC, or IFN gene signature levels, or a combination thereof, in a subject. In aspects, changes in blood MxA levels, cytokines, chemokines, or other proteins (e.g., IFN-α, IL-17, and IFN-γ), PBMC, or IFN gene signature levels, or a combination thereof, can be associated with the pathogenesis of myositis or the effect of anti-ILT7 antibodies or antigen-binding fragments thereof. Suitable assays for investigating changes in blood MxA levels, cytokines, chemokines, or other proteins (e.g., IFN-α, IL-17, and IFN-γ), PBMC, or IFN gene signature levels, or combinations thereof, can include flow cytometry, histology, immunohistochemistry, blood analysis, microscopy, PCR, ELISA, and combinations thereof.

[0097] In aspects, the anti-ILT7 antibody or antigen-binding fragment thereof can achieve at least about a 10%, 15%, 20%, 25%, 50%, 60%, 70%, 80%, 90%, 95%, 96%, 97%, 98%, 99%, or up to about a 100% reduction in pDC levels compared to an otherwise equivalent method in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof. The reduction or elimination of pDC, blood MxA levels, cytokine, chemokine, or other protein (e.g., IFN-α, IL-17, and IFN-γ), PBMC, or IFN gene signature levels, or a combination thereof, can be sustained over time. In aspects, the depletion of pDCs, blood MxA levels, cytokines, chemokines, or other proteins (e.g., IFN-α, IL-17, and IFN-γ), PBMCs, or IFN gene signature levels, or a combination thereof, can last for at least 1 day, at least 2 days, at least 3 days, at least 4 days, at least 5 days, at least 6 days, at least 7 days, at least 8 days, at least 9 days, at least 10 days, at least 15 days, at least 20 days, at least 25 days, or at least 30 days. In another embodiment, the depletion of pDCs, blood MxA levels, cytokines, chemokines, or other proteins (e.g., IFN-α, IL-17, and IFN-γ), PBMCs, or IFN gene signature levels, or a combination thereof, can last for at least 1 week, at least 2 weeks, at least 3 weeks, at least 4 weeks, at least 5 weeks, at least 6 weeks, at least 7 weeks, at least 8 weeks, at least 9 weeks, or at least 10 weeks. In aspects, depletion of pDCs, blood MxA levels, cytokines, chemokines, or other proteins (e.g., IFN-α, IL-17, and IFN-γ), PBMCs, or IFN gene signature levels, or combinations thereof, can last for at least 1 month, at least 2 months, at least 3 months, at least 4 months, at least 5 months, at least 6 months, at least 7 months, at least 8 months, at least 9 months, at least 10 months, at least 11 months, or at least 12 months.

[0098] In embodiments, pharmacodynamic studies for an anti-ILT7 antibody or antigen-binding fragment thereof are performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 days, 18 days, 19 days, 20 days, 21 days, 22 days, 23 days, 24 days, 25 days, 26 days, 27 days, 28 days, 29 days, 30 days, 31 days, 32 days, 33 days, 34 days, 35 days, 36 days, 37 days, 38 days, 39 days, 40 days, 41 days, 42 days, 43 days, 44 days, 45 days, 46 days, 47 days, 48 ​​days, 49 days, 50 days, 51 days, 52 days, 53 days, 54 days, 55 days, 56 days, 57 days, 58 days, 59 days, 60 days, 61 days, 62 days, 63 days, 64 days, 65 days, 66 days, 67 days The study can be conducted at 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, pharmacodynamic studies for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days after initiation of treatment.

[0099] Health Assessment Questionnaire Disability Index (HAQ-DI) In embodiments, the survey comprises the HAQ-DI. The HAQ-DI assesses the physical function of a subject in need of treatment (HAQ-DI Form 05a). In embodiments, the HAQ-DI can be scored at about 0, 1, 2, or up to about 3. In embodiments, the HAQ-DI score is at least about 0, about 1, about 2%, or about 3%. In embodiments, the HAQ-DI can include eight sections: dressing and grooming, getting up, eating, walking, hygiene, reaching, grasping, and general daily activities. In embodiments, each section is scored from 0 (no difficulty) to 3 (unable to perform). In embodiments, the HAQ-DI survey can include assessing subjects in need of treatment who are administered an anti-ILT7 antibody or antigen-binding fragment thereof compared to a baseline. In embodiments, the subject's HAQ-DI score is reduced compared to an otherwise equivalent procedure in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof.

[0100] In embodiments, the HAQ-DI survey for an anti-ILT7 antibody or antigen-binding fragment thereof is administered at about day -28, day -27, day -26, day -25, day -24, day -23, day -22, day -21, day -20, day -19, day -18, day -17, day -16, day -15, day -14, day -13, day -12, day -11, day -10, day -9, day -8, day -7, day -6, day -5, day -4, day -3, day -2, day -1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 1 week after initiation of treatment. , 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, the HAQ-DI survey for an anti-ILT7 antibody or antigen-binding fragment thereof is performed at about day -28, day -27, day -26, day -25, day -24, day -23, day -22, day -21, day -20, day -19, day -18, day -17, day -16, day -15, day -14, day -13, day -12, day -11, day -10 after initiation of treatment. , day -9, day -8, day -7, day -6, day -5, day -4, day -3, day -2, day -1, day 0, day 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days.

[0101] Bilateral Manual Muscle Testing 8 (MMT8) In embodiments, the study includes bilateral MMT8. In embodiments, bilateral MMT8 is a series of eight designated muscles tested bilaterally (MMT8, 2007). In embodiments, because axial (neck flexor) testing is included, the potential score for MMT8 is at least up to about 150. In embodiments, bilateral MMT8 is a score of about 0, 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 81, 82, 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, 97, 98, 99, 100, 101, 102, 103, 104, 105, 106, 107, 108, 109, 110, 111, 112, 113, 114, 115, 116, 117, 118, 119, 120, 121, 122 2, 123, 124, 125, 126, 127, 128, 129, 130, 131, 132, 133, 134, 135, 136, 137, 138, 139, 140, 141, 142, 143, 144, 145, 146, 147, 148, 149, or about 150. In embodiments, bilateral MMT8 is about 0-10, about 10-30, about 20-40, about 30-50, about 40-60, about 50-70, about 60-80, about 70-90, about 80-100, about 90-110, about 100-120, about 110-130, about 120-140, or about 130-150. In embodiments, the bilateral MMT8 study can include studying a subject in need of treatment who is administered an anti-ILT7 antibody or antigen-binding fragment thereof, compared to a baseline. In embodiments, the subject's bilateral MMT8 score is increased compared to an otherwise equivalent approach in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0102] In embodiments, bilateral MMT8 surveys for an anti-ILT7 antibody or antigen-binding fragment thereof are performed on about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −5, day −6, day −7, day −8, day −9, day −10, day −11, day −12, day −13, day −14, day −15, day −16, day −17, day −18, day −19, day −20, day −21, day −22, day −23, day −24, day −25, day −26, day −27, day −28, day −29, day −30, day −31, day −32, day −33, day −34, day −35, day −36, day −37, day −38, day −39, day −40, day −41, day −42, day −43, day −44, day −45, day −46, day −47, day −48, day −49, day −50, day −51, day −52, day −53, day −54, day −55, day −56, day −57, day −58, day −59, day −60, day −61, day −62, day −63, day −64, day −65, day −66, day −67, day −68, day −69, day −70, day −71, day −72, day −73, day −74, day −75, day −76, day −77, day −78 The study may be conducted at weeks 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, or at least about 56 weeks. In embodiments, bilateral MMT8 surveys for an anti-ILT7 antibody or antigen-binding fragment thereof are performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10 after initiation of treatment. , day -9, day -8, day -7, day -6, day -5, day -4, day -3, day -2, day -1, day 0, day 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days.

[0103] Myositis Disease Activity Assessment Tool (MDAAT) and Patient Total Disease Activity (PtGDA) In embodiments, the survey includes a total MDAAT. The total MDAAT measures the degree of disease activity in extramuscular organ systems and muscles (MDAAT, 2005). In embodiments, the total MDAAT can include, but is not limited to, a physician-performed global disease activity (PhGDA) survey and an extramuscular total survey. The PhGDA is a physician's assessment of overall disease activity (extramuscular organ systems and muscle disease activity) based on clinical laboratory surveys. In embodiments, the total MDAAT can be scored from about 0, about 1, about 2, about 3%, and up to about 4. In embodiments, the total MDAAT is scored from about 0 to 4, or from about 1 to 4. In embodiments, the PhGDA can be scored using a 10-cm VAS ranging from 0 (no evidence of disease activity) to 4 (very severe disease activity). In embodiments, the total extramuscular survey assesses disease activity in all extramuscular systems (excluding muscle disease activity) using a 10 cm VAS ranging from 0 (no evidence of disease activity) to 4 (very severe disease activity). In embodiments, the total MDAAT survey uses an activity index ranging from 0 ("absent in the last 4 weeks") to 4 ("new in the last 4 weeks"). In embodiments, the total MDAAT survey can include surveying subjects in need of treatment who are administered an anti-ILT7 antibody or antigen-binding fragment thereof compared to baseline. In embodiments, a subject in need of treatment who is administered an anti-ILT7 antibody or antigen-binding fragment thereof can be considered in complete remission if they have no evidence of disease activity during the study (i.e., a PhGDA VAS of 0).

[0104] In aspects, the subject's total MDAAT score is reduced compared to an otherwise equivalent method in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof.

[0105] In embodiments, the MDAAT survey for an anti-ILT7 antibody or antigen-binding fragment thereof is performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 1 week after initiation of treatment. , 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, the MDAAT survey for an anti-ILT7 antibody or antigen-binding fragment thereof is performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −15, day −16, day −17, day −18, day −19, day −20, day −21, day −22, day −23, day −24, day −25, day −26, day −27, day −28, day −29, day −30, day −31, day −32, day −33, day −34, day −35, day −36, day −37, day −38, day −39, day −40, day −41, day −42, day −43, day −44, day −45, day −46, day −47, day −48, day −49, day −50, day −51, day −52, day −53, day −54, day −55, day −56, day −57, day −58, day −59, day −60, day −61, day −62, day −63, day −64, day −65, day −66, day −67, day −68, day −69, day −70, day −71, day −72, day −73, day −74, day −75, day −76, day −77, day −78, day −79, day −80, day −81, day −82, day −83, day −84, day −85, day −86, day −87, day −88, day −89, day −90, day −91, day −92, Surveys can be conducted on days -9, -8, -7, -6, -5, -4, -3, -2, -1, 0, 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days.

[0106] In embodiments, the survey includes a Patient Total Disease Activity (PtGDA). The PtGDA measures a patient-wide survey of disease activity at the time of the survey (PtGDA Form 03). In embodiments, the PtGDA survey is scored from about 1, 2, 3, 4, 5, 6, 7, 8, 9, up to about 10. In embodiments, the PtGDA survey is scored from about 0 to 10, about 1 to 5, about 4 to 10, or about 1 to 10. In embodiments, the PtGDA can be measured using a 10 cm visual analog scale (VAS), ranging from "no evidence of disease activity" to "very active or severe disease activity." In embodiments, the PtGDA survey can include surveying a subject in need of treatment to be administered an anti-ILT7 antibody or antigen-binding fragment thereof, compared to a baseline. In embodiments, the subject's PtGDA score is reduced compared to an otherwise equivalent method in which the subject is not administered the anti-ILT7 antibody, or antigen-binding fragment thereof.

[0107] In embodiments, the PtGDA study for an anti-ILT7 antibody or antigen-binding fragment thereof is performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 1 week after initiation of treatment. , 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, PtGDA studies for an anti-ILT7 antibody or antigen-binding fragment thereof are performed on about day −28, −27, −26, −25, −24, −23, −22, −21, −20, −19, −18, −17, −16, −15, −14, −13, −12, −11, −10, or 124 days after initiation of treatment. Surveys can be conducted on days -9, -8, -7, -6, -5, -4, -3, -2, -1, 0, 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days.

[0108] Muscle-related enzymes In embodiments, the investigation involves measuring muscle-related enzyme levels. Muscle-related enzymes investigated may include, but are not limited to, alanine transaminase (ALT), aspartate transaminase (AST), lactate dehydrogenase (LDH), creatine kinase (CK), and aldolase, and combinations thereof. In embodiments, whole blood, plasma, serum, and urine are collected to investigate muscle-related enzyme levels. In embodiments, investigating muscle-related enzyme levels may involve investigating a subject in need of treatment to which an anti-ILT7 antibody or antigen-binding fragment thereof is administered, compared to a baseline. In embodiments, administration of the anti-ILT7 antibody or antigen-binding fragment thereof is effective in reducing muscle-related enzyme levels in the subject by at least about, or by up to about 50%, 55%, 60%, 65%, 70%, 75%, 80%, 85%, 90%, 95%, 96%, 97%, 98%, 99%, or up to about 100% of pre-administration levels. In aspects, administration of the anti-ILT7 antibody or antigen-binding fragment thereof is effective in reducing muscle-associated enzyme levels in a subject by at least about, or by up to about: 3% to 5%, 5% to 10%, 10% to 20%, or 5% to 25%, compared to baseline levels before administration.

[0109] In an embodiment, the investigation of muscle-associated enzyme levels in response to an anti-ILT7 antibody or antigen-binding fragment thereof is performed at about day -28, day -27, day -26, day -25, day -24, day -23, day -22, day -21, day -20, day -19, day -18, day -17, day -16, day -15, day -14, day -13, day -12, day -11, day -10, day -9, day -8, day -7, day -6, day -5, day -4, day -3, day -2, day -1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day , 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, the levels of muscle-associated enzymes in response to an anti-ILT7 antibody or antigen-binding fragment thereof are examined at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −14, day −15, day −16, day −17, day −18, day −19, day −20, day −21, day −22, day −23, day −24, day −25, day −26, day −27, day −28, day −29, day −30, day −31, day −32, day −33, day −34, day −35, day −36, day −37, day −38, day −39, day −40, day −41, day −42, day −43, day −44, day −45, day −46, day −47, day −48, day −49, day −50, day −51, day −52, day −53, day −54, day −55, day −56, day −57, day −58, day −59, day −60, day −61, day −62, day −63, day −64, day −65, day −66, day −67, day −68, day −69, day −70, day −71, day −72, day −73, day −74, day −75, day −76, day −77, day −78, day −79, day −80, day −81, day −82, day −83, day −84, day −85, day −86, day −87, day −88, day −89, day −90, day −9 Surveys can be conducted on days 0, -9, -8, -7, -6, -5, -4, -3, -2, -1, 0, 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days.

[0110] Dermatomyositis Disease Area and Severity Index (CDASI) In some embodiments, the survey includes a CDASI measurement. The CDASI measurement can be used to assess the severity of cutaneous DM and detect improvements in disease activity. In some embodiments, the CDASI is about 0, 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 81, 82, 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, 97, 98, 99, 100, 101, 102, 103, 104, 105, 106, 107, 108, 109, 110, 111, 112, 113, 114 9, 91, 92, 93, 94, 95, 96, 97, 98, 99, and up to about 100. In embodiments, the CDASI is scored from about 0 to 10, about 1 to 10, about 0 to 30, about 1 to 30, about 10 to 30, about 0 to 40, about 1 to 40, about 20 to 40, about 30 to 50, about 40 to 60, about 50 to 70, about 60 to 80, about 70 to 90, about 0 to 100, about 1 to 100, or about 80 to 100. The CDASI activity scale can range from 0 to 100, and the damage scale can range from 0 to 32. In embodiments, the CDASI scale can rate disease improvement in 15 different body regions using three activity (erythema, scale, and erosion / ulceration) and two damage (poikiloderma and calcinosis) scales. In embodiments, CDASI measurements can examine the presence and severity of Gottron papules on the hands, periungual changes, alopecia, or a combination thereof. In embodiments, CDASI examination can include examining a subject in need of treatment who is administered an anti-ILT7 antibody or antigen-binding fragment thereof, compared to a baseline. In embodiments, the subject's CDASI score is reduced compared to an otherwise equivalent treatment in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0111] In embodiments, CDASI measurements for an anti-ILT7 antibody or antigen-binding fragment thereof are measured at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 1 week after initiation of treatment. , 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, CDASI measurements for an anti-ILT7 antibody or antigen-binding fragment thereof can be taken at about day 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0112] Myositis Damage Index (MDI) In embodiments, the survey includes an MDI survey. The MDI survey can be used to survey the extent of disease damage across all time systems (MDI Form 08, 2001). In embodiments, the MDI survey can include a series of organ-specific questions regarding the presence or absence of given signs, symptoms, or problems to measure the extent of damage. In embodiments, the MDI survey is scored from about 0, 1, 2, 3, up to about 4. In embodiments, the MDI survey is scored from about 0 to 4, or from about 1 to 4. In embodiments, the MDI survey is scored using a 10-cm VAS to measure the severity of damage, from 0 (“no evidence of disease”) to 4 (“extreme or maximal disease damage”). In embodiments, the MDI survey can include surveying subjects in need of treatment who are administered an anti-ILT7 antibody or antigen-binding fragment thereof, compared to a baseline. In embodiments, the subject's MDI score is reduced compared to an otherwise equivalent method in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0113] In embodiments, the MDI survey for the anti-ILT7 antibody or antigen-binding fragment thereof is conducted at about day -28, day -27, day -26, day -25, day -24, day -23, day -22, day -21, day -20, day -19, day -18, day -17, day -16, day -15, day -14, day -13, day -12, day -11, day -10, day -9, day -8, day -7, day -6, day -5, day -4, day -3, day -2, day -1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 1 week, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day 70, day 71, The study can be conducted at 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, MDI surveys for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, 24 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0114] Myositis Functional Index 3 (FI3) In embodiments, the survey includes a Myositis FI3 survey. The Myositis FI3 survey can be used to assess functional outcomes of muscle endurance and function of frequently affected muscle groups (MFI3 2016). In embodiments, the Myositis FI3 survey is scored from about 1, 2, 3, 4, 5, 6, 7, 8, 9, up to about 10. In embodiments, the Myositis FI3 survey is scored from about 0 to 10, about 1 to 5, about 4 to 10, or about 1 to 10. In embodiments, the Myositis FI3 score ranges from 0 (no exertion) to 10 (maximum exertion). In embodiments, the Myositis FI3 survey is scored as the number of repetitions performed correctly, with a maximum number of 60 or 120 repetitions, depending on the muscle group surveyed. In embodiments, the Myositis FI3 survey can include assessing a subject in need of treatment to which an anti-ILT7 antibody or antigen-binding fragment thereof is administered, compared to a baseline. In aspects, the subject's Myositis FI3 score is reduced compared to an otherwise equivalent method in which the subject is not administered the anti-ILT7 antibody, or antigen-binding fragment thereof.

[0115] In embodiments, the Myositis FI3 survey for an anti-ILT7 antibody or antigen-binding fragment thereof is performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 1 week after initiation of treatment. , 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, Myositis FI3 studies for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about day 1, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0116] forced vital capacity (FVC) In embodiments, the investigation includes an FVC measurement. The FVC measurement can be used to investigate pulmonary function (e.g., lung compliance). In embodiments, the FVC measurement measures the amount of air that can be forcibly exhaled from the lungs of a subject in need of treatment. In embodiments, an increase in the FVC measurement (e.g., lung compliance) comprises an increase in pulmonary vascularity. In embodiments, the FVC measurement can include investigating a subject in need of treatment to which an anti-ILT7 antibody or antigen-binding fragment thereof is administered compared to a baseline. In embodiments, the subject's FVC measurement is increased compared to an otherwise equivalent procedure in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof.

[0117] In embodiments, FVC measurements for an anti-ILT7 antibody or antigen-binding fragment thereof are measured at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, 56 weeks, 57 weeks, 58 weeks, 59 weeks, 60 weeks, 61 weeks, 62 weeks, 63 weeks, 64 weeks, 65 weeks, 66 weeks, 67 weeks, 6 The study can be conducted at 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, FVC measurements for an anti-ILT7 antibody or antigen-binding fragment thereof can be taken at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0118] Skindex16 score In embodiments, the survey includes an Skindex 16 score. The Skindex 16 score can be used to rate skin conditions experienced over the past week. In embodiments, the Skindex 16 can be scored from about 0, 1, 2, 3, 4, 5, and up to about 6. In embodiments, the Skindex 16 score is a 16-item survey using a scale from 0 ("not bothered at all") to 6 ("always bothered"). In embodiments, the Skindex 16 score is at least about 0, about 1, about 2, about 3, about 4, about 5, or about 6. In embodiments, these scores are categorized into three subscales: symptomatic, emotional, and functional. In embodiments, the Skindex 16 score can be compared to a baseline to assess a subject in need of treatment to whom an anti-ILT7 antibody or antigen-binding fragment thereof is administered. In embodiments, the subject's Skindex16 score is reduced compared to an otherwise equivalent regimen in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof (Atherton et al., Expert. Opin. Drug Saf., 2009).

[0119] In embodiments, the Skindex16 score for an anti-ILT7 antibody or antigen-binding fragment thereof is measured at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day 70, day 71, day 7 The study can be conducted at 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, the Skindex16 score for an anti-ILT7 antibody or antigen-binding fragment thereof can be assessed at about day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0120] 36-item short form (SF36) survey In embodiments, the investigation comprises the SF36 survey. The SF36 survey can measure a subject's functional health and well-being with questions across eight health domains: physical functionality, role-physical, bodily pain, overall health, vitality, social functionality, role-emotional, and mental health. In embodiments, the SF36 survey can comprise surveying a subject in need of treatment to which an anti-ILT7 antibody or antigen-binding fragment thereof is administered compared to a baseline. In embodiments, the subject's functional health and well-being is improved compared to an otherwise equivalent approach in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof.

[0121] In embodiments, the SF36 survey for an anti-ILT7 antibody or antigen-binding fragment thereof is administered at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day 70, day 71, day 7 The study may be conducted at weeks 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, or at least about 56 weeks. In embodiments, SF36 surveys for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0122] Patient-Reported Outcomes Measurement Information System (PROMIS) Fatigue 8a In embodiments, the survey comprises the PROMIS Fatigue 8a Survey. The PROMIS Fatigue 8a Survey surveys a wide range of self-reported symptoms, from a mild subjective feeling of tiredness to an overwhelming, debilitating, and persistent feeling of exhaustion. In embodiments, fatigue is divided into the experience of fatigue (frequency, duration, and intensity) and the impact of fatigue on physical, mental, and social activities. In embodiments, the PROMIS Fatigue 8a Survey surveys symptoms over the past week. In embodiments, the PROMIS Fatigue 8a Survey can include surveying subjects in need of treatment who are administered an anti-ILT7 antibody or antigen-binding fragment thereof compared to a baseline. In embodiments, the subject's self-reported symptoms are improved compared to an otherwise comparable approach in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0123] In embodiments, a PROMIS Fatigue 8a survey for an anti-ILT7 antibody or antigen-binding fragment thereof is administered at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day , 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, the PROMIS Fatigue 8a survey for an anti-ILT7 antibody or antigen-binding fragment thereof can be administered at about day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after treatment initiation.

[0124] PROMIS Physical Function 8b In embodiments, the survey includes PROMIS Physical Function 8b. The PROMIS Physical Function 8b survey can survey a wide range of self-reported abilities. The PROMIS Physical Function 8b survey can include functionality of a subject's upper extremities (e.g., dexterity), lower extremities (e.g., walking or mobility), and central areas (e.g., neck and back), as well as daily activities (e.g., running errands). In embodiments, the PROMIS Physical Function 8b survey can include surveying a subject in need of treatment who is administered an anti-ILT7 antibody or antigen-binding fragment thereof, compared to a baseline. In embodiments, the subject's self-reported ability is improved compared to an otherwise equivalent approach in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0125] In embodiments, a PROMIS fatigue 8b survey for an anti-ILT7 antibody or antigen-binding fragment thereof is administered at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day , 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, the PROMIS Physical Function 8b survey for an anti-ILT7 antibody or antigen-binding fragment thereof can be administered at about day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0126] PROMIS Pain Interference 6a In embodiments, the survey comprises a PROMIS Pain Interference 6a Survey. The PROMIS Pain Interference 6a Survey can survey the consequences of pain over a subject's lifetime. The PROMIS Pain Interference 6a Survey can include the extent to which pain interferes with engaging in social, cognitive, emotional, physical, and restful activities. In embodiments, the PROMIS Pain Interference 6a Survey can comprise surveying a subject in need of treatment to which an anti-ILT7 antibody or antigen-binding fragment thereof is administered compared to a baseline. In embodiments, the subject's pain disorder is improved compared to an otherwise comparable treatment in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0127] In embodiments, a PROMIS pain interference 6a survey for an anti-ILT7 antibody or antigen-binding fragment thereof is administered on about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6 after initiation of treatment. The study can be conducted at 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, PROMIS pain interference 6a surveys for an anti-ILT7 antibody or antigen-binding fragment thereof can be surveyed at about day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0128] PROMIS Dyspnea Severity Survey In embodiments, the survey comprises a PROMIS Dyspnea Severity Survey. The PROMIS Dyspnea Severity Survey can survey the severity of dyspnea in subjects experiencing response to various activities (e.g., dressing themselves unaided, preparing a meal, walking 20 flights of stairs). In embodiments, the PROMIS Dyspnea Severity Survey can comprise surveying subjects in need of treatment who are administered an anti-ILT7 antibody or antigen-binding fragment thereof compared to a baseline. In embodiments, the subject's severity of dyspnea is improved compared to an otherwise equivalent approach in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0129] In embodiments, the PROMIS Dyspnea Severity Survey for an anti-ILT7 antibody or antigen-binding fragment thereof is administered at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day 70, day The study can be conducted at day 1, week 2, week 3, week 4, week 5, week 6, week 7, week 8, week 9, week 10, week 11, week 12, week 13, week 14, week 15, week 16, week 17, week 18, week 19, week 20, week 21, week 22, week 23, week 24, week 25, week 26, week 27, week 28, week 29, week 30, week 31, week 32, week 33, week 34, week 35, week 36, week 37, week 38, week 39, week 40, week 41, week 42, week 43, week 44, week 45, week 46, week 47, week 48, week 49, week 50, week 51, week 52, week 53, week 54, week 55, or at least about week 56. In embodiments, the PROMIS dyspnea severity survey for an anti-ILT7 antibody or antigen-binding fragment thereof can be administered at about day 1, 4 weeks ± 3 days, 12 weeks ± 3 days, 24 weeks ± 3 days, 36 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0130] 24-hour Numeric Rating Scale (NRS) survey of total pain In embodiments, the survey includes a 24-hour total pain NRS survey. The 24-hour total pain NRS survey can survey the intensity of pain a subject may have experienced over the past 24 hours. In embodiments, the 24-hour total pain NRS survey can be scored from about 1, 2, 3, 4, 5, 6, 7, 8, 9, and up to about 10. In embodiments, the 24-hour total pain NRS survey is about 0-5, about 0-10, or about 1-10. The 24-hour total pain NRS survey scale can range from 0 (no pain) to 10 (worst pain imaginable). In embodiments, the 24-hour total pain NRS survey can include surveying a subject in need of treatment who is administered an anti-ILT7 antibody or antigen-binding fragment thereof compared to a baseline. In embodiments, the subject's score is reduced compared to an otherwise equivalent treatment in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0131] In embodiments, a 24-hour NRS survey of total pain for an anti-ILT7 antibody or antigen-binding fragment thereof is administered at about day −28, −27, −26, −25, −24, −23, −22, −21, −20, −19, −18, −17, −16, −15, −14, −13, −12, −11, −10, −9, −8, −7, −6, −5, −4, −3, −2, −1, 0, 1, 2, 3, 4, 5, 6, or 7 days after initiation of treatment. The study can be conducted at 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, a 24-hour NRS survey of total pain for an anti-ILT7 antibody or antigen-binding fragment thereof is administered at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −14, day −15, day −16, day −17, day −18, day −19, day −20, day −21, day −22, day −23, day −24, day −25, day −26, day −27, day −28, day −29, day −30, day −31, day −32, day −33, day −34, day −35, day −36, day −37, day −38, day −39, day − Surveys can be conducted on days 0, -9, -8, -7, -6, -5, -4, -3, -2, -1, 0, 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days.

[0132] Patient Global Impression of Change (PGIC) Survey In embodiments, the investigation includes a PGIC survey. The PGIC survey uses a categorical scale to ask the subject how much their myositis has changed. In embodiments, the PGIC survey can be administered to a subject in need of treatment after administration of an anti-ILT7 antibody or antigen-binding fragment thereof of the present disclosure. In embodiments, the PGIC survey score is at least about 1, about 2, about 3, about 4, or about 5. In embodiments, the PGIC survey scale can range from 1 (much better) to 5 (much worse). In embodiments, the level of the PGIC survey is reduced by at least about 1, about 2, about 3, or about 4 points after treatment with a composition provided herein. In embodiments, the PGIC survey can include surveying a subject in need of treatment who is administered an anti-ILT7 antibody or antigen-binding fragment thereof compared to a baseline. In embodiments, the subject's score is reduced compared to an otherwise equivalent method in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0133] In embodiments, the PGIC survey for an anti-ILT7 antibody or antigen-binding fragment thereof is performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 1 week after initiation of treatment. , 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, PGIC surveys for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days after initiation of treatment.

[0134] Total Improvement Score (TIS) based on six Core Set Scales (CSM) In embodiments, the survey includes the TIS, which is a composite score that includes surveys of the Health Examination Questionnaire Disability Index (HAQ-DI), Patient Total Disease Activity (PtGDA), Physician Total Disease Activity (PhGDA), bilateral Manual Muscle Test 8 (MMT8), laboratory muscle enzymes, and extramuscular total survey (MRC 2016). In embodiments, the TIS is about 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, The TIS can be scored as 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 81, 82, 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, 97, 98, 99, and up to about 100. In embodiments, the TIS is about 1 to 10, about 10 to 30, about 20 to 40, about 30 to 50, about 40 to 60, about 50 to 70, about 60 to 80, about 70 to 90, or about 80 to 100. The TIS can be 20 or greater, or 40 or greater. In embodiments, the baseline can be used as a continuous outcome measure using the TIS. In embodiments, the baseline can be used as a categorical outcome of improvement (minimal, moderate, or large improvement). In embodiments, the baseline can be used as both a continuous outcome measure and a categorical outcome of improvement, thus it is a hybrid measure. In embodiments, a change in the baseline is measured. In embodiments, a subject's score is increased compared to an otherwise equivalent approach in which the subject is not administered the anti-ILT7 antibody or antigen-binding fragment thereof.

[0135] In embodiments, the TIS survey for an anti-ILT7 antibody or antigen-binding fragment thereof is conducted at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 days, 18 days, 19 days, 20 days, 21 days, 22 days, 23 days, 24 days, 25 days, 26 days, 27 days, 28 days, 29 days, 30 days, 31 days, 32 days, 33 days, 34 days, 35 days, 36 days, 37 days, 38 days, 39 days, 40 days, 41 days, 42 days, 43 days, 44 days, 45 days, 46 days, 47 days, 48 ​​days, 49 days, 50 days, 51 days, 52 days, 53 days, 54 days, 55 days, 56 days, 57 days, 58 days, 59 days, 60 days, 61 days, 62 days, 63 days, 64 days, 65 days, 66 days, 67 days, The study can be conducted at 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, the TIS survey for an anti-ILT7 antibody or antigen-binding fragment thereof is performed on about day -28, day -27, day -26, day -25, day -24, day -23, day -22, day -21, day -20, day -19, day -18, day -17, day -16, day -15, day -14, day -13, day -12, day -11, day -10, day -11, day -12, day -13, day -14, day -15, day -16, day -17, day -18, day -19, day -20, day -21, day -22, day -23, day -24, day -25, day -26, day -27, day -28, day -29, day -30, day -31, day -32, day -33, day -34, day -35, day -36, day -37, day -38, day -39, day -40, day -41, day -42, day -43, day -44, day -45, day -46, day -47, day -48, day -49, day -50, day -51, day -52, day -53, day -54, day -55, day -56, day -57, day -58, day -59, day -60, day -61, day -62, day -63, day -64, day -65, day -66, day -67, day -68, day -69, day -70, day -71, day -72, day -73, day -74, day -75, day -76, day -77, day -78, day -79, day -80, day -81, day -82, day -83, day -84, day -85, day -86, day -87, day -88, day Surveys can be conducted on days 9, -8, -7, -6, -5, -4, -3, -2, -1, 0, 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, 48 ​​weeks ± 3 days, and 56 weeks ± 7 days.

[0136] 28 joints In embodiments, the survey includes a 28-joint count. Tenderness and swelling of 28 specified joints are assessed. In embodiments, the 28 specified joints include, but are not limited to, the left and right elbows, wrists, metacarpophalangeal (MCP) 1, MCP2, MCP3, MCP4, MCP5, proximal interphalangeal (PIP) 1, PIP2, PIP3, PIP4, and PIP5 joints of the upper extremities, and the left and right knees of the lower extremities. In embodiments, the change in the 28-joint count can be measured relative to a baseline. In embodiments, the subject's score is improved compared to an otherwise equivalent method in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0137] In embodiments, the 28-joint count survey for an anti-ILT7 antibody or antigen-binding fragment thereof is performed at about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 1 week after initiation of treatment. , 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, a 28-joint count survey for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about day 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0138] Actigraphy In embodiments, the assessment comprises actigraphy. Actigraphy is a measure of overall activity and sleep patterns, including sleep duration and fragmentation. In embodiments, the activity monitoring device is an ActiGraph, a wearable watch device. In embodiments, changes in overall activity and sleep patterns are measured in subjects administered an anti-ILT7 antibody or antigen-binding fragment thereof, compared to a baseline. In embodiments, the subject's score is improved compared to an otherwise equivalent method in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0139] In embodiments, actigraphy testing for the anti-ILT7 antibody or antigen-binding fragment thereof is performed on or about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −5, day −6, day −7, day −8, day −9, day −10, day −11, day −12, day −13, day −14, day −15, day −16, day −17, day −18, day −19, day −20, day −21, day −22, day −23, day −24, day −25, day −26, day −27, day −28, day −29, day −30, day −31, day −32, day −33, day −34, day −35, day −36, day −37, day −38, day −39, day −40, day −41, day −42, day −43, day −44, day −45, day −46, day −47, day −48, day −49, day −50, day −51, day −52, day −53, day −54, day −55, day −56, day −57, day −58, day −59, day −60, day −61, day −62, day −63, day −64, day −65, day −66, day −67, day −68, day −69, day −70, day −71, day −72, day −73, day −74, day −75, day −76, day −77, day −78, day The study may be conducted at weeks 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, or at least about 56 weeks. In embodiments, actigraphy studies for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about day 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0140] Handheld Dynamometry In embodiments, the survey includes handheld dynamometry. In embodiments, handheld dynamometry can be used to assess muscle strength, particularly for assessing the strength of a single muscle group (Baschung et al., 2018), and has demonstrated robust reliability, construct validity, and responsiveness in myositis patients (Saygin et al., 2021). Handheld dynamometry can be used to assess muscle strength. In embodiments, the handheld dynamometry survey can assess the strength of a single muscle group at a time. In embodiments, the handheld dynamometry survey of a subject administered an anti-ILT7 antibody or antigen-binding fragment thereof is measured relative to a baseline. In embodiments, the subject's score is improved compared to an otherwise comparable method in which the subject is not administered an anti-ILT7 antibody or antigen-binding fragment thereof.

[0141] In embodiments, handheld dynamometry surveys for an anti-ILT7 antibody or antigen-binding fragment thereof are performed on about day −28, day −27, day −26, day −25, day −24, day −23, day −22, day −21, day −20, day −19, day −18, day −17, day −16, day −15, day −14, day −13, day −12, day −11, day −10, day −9, day −8, day −7, day −6, day −5, day −4, day −3, day −2, day −1, day 0, day 1, day 2, day 3, day 4, day 5, day 6, day 7, day 8, day 9, day 10, day 11, day 12, day 13, day 14, day 15, day 16, day 17, day 18, day 19, day 20, day 21, day 22, day 23, day 24, day 25, day 26, day 27, day 28, day 29, day 30, day 31, day 32, day 33, day 34, day 35, day 36, day 37, day 38, day 39, day 40, day 41, day 42, day 43, day 44, day 45, day 46, day 47, day 48, day 49, day 50, day 51, day 52, day 53, day 54, day 55, day 56, day 57, day 58, day 59, day 60, day 61, day 62, day 63, day 64, day 65, day 66, day 67, day 68, day 69, day 70, day 71, , 1 week, 2 weeks, 3 weeks, 4 weeks, 5 weeks, 6 weeks, 7 weeks, 8 weeks, 9 weeks, 10 weeks, 11 weeks, 12 weeks, 13 weeks, 14 weeks, 15 weeks, 16 weeks, 17 weeks, 18 weeks, 19 weeks, 20 weeks, 21 weeks, 22 weeks, 23 weeks, 24 weeks, 25 weeks, 26 weeks, 27 weeks, 28 weeks, 29 weeks, 30 weeks, 31 weeks, 32 weeks, 33 weeks, 34 weeks, 35 weeks, 36 weeks, 37 weeks, 38 weeks, 39 weeks, 40 weeks, 41 weeks, 42 weeks, 43 weeks, 44 weeks, 45 weeks, 46 weeks, 47 weeks, 48 ​​weeks, 49 weeks, 50 weeks, 51 weeks, 52 weeks, 53 weeks, 54 weeks, 55 weeks, or at least about 56 weeks. In embodiments, handheld dynamometry studies for an anti-ILT7 antibody or antigen-binding fragment thereof can be conducted at about day 1, 4 weeks ± 3 days, 8 weeks ± 3 days, 12 weeks ± 3 days, 16 weeks ± 3 days, 20 weeks ± 3 days, 24 weeks ± 3 days, 28 weeks ± 3 days, 32 weeks ± 3 days, 36 weeks ± 3 days, 40 weeks ± 3 days, 44 weeks ± 3 days, and 48 weeks ± 3 days after initiation of treatment.

[0142] Pharmaceutical Composition In embodiments, pharmaceutical compositions are provided. The pharmaceutical composition can comprise an anti-ILT7 antibody or antigen-binding fragment thereof. In embodiments, the pharmaceutical composition is part of a treatment regimen comprising an anti-ILT7 antibody or antigen-binding fragment thereof and one or more additional therapeutic agents provided herein. In embodiments, the one or more additional therapeutic agents can comprise a corticosteroid, a DMARD, or an immunomodulatory agent, or a combination thereof.

[0143] Many drugs can be administered orally as liquids, capsules, tablets, or chewable tablets. The oral route is the most convenient, usually the safest, and least expensive, and therefore the most frequently used. However, it has limitations due to the way drugs typically move through the digestive tract. For orally administered drugs, absorption may begin in the mouth and stomach. However, most drugs are typically absorbed from the small intestine. Drugs pass through the intestinal wall and travel to the liver, and are then transported to their target site via the bloodstream. The intestinal wall and liver chemically alter (metabolize) many drugs, reducing the amount of drug that reaches the bloodstream. As a result, these drugs are often administered in smaller doses when injected intravenously to achieve the same effect.

[0144] In the subcutaneous route, a needle is inserted into the fatty tissue just below the skin. After administration of a drug (e.g., an anti-ILT7 antibody or its antigen-binding fragment), the drug travels to small blood vessels (capillaries) and is carried by the bloodstream. Alternatively, the drug reaches the bloodstream through lymphatic vessels. The intramuscular route is preferred over the subcutaneous route when larger amounts of medication are needed. A longer needle is used because the muscle is beneath the skin and fatty tissue. Drugs are usually injected into the muscles of the abdomen (avoiding a two-inch radius around the navel), upper arm, thigh, or buttocks. The rate at which a drug is absorbed into the bloodstream depends in part on the blood supply to the muscle. The smaller the blood supply, the more time it takes for the drug to be absorbed. In the intravenous route, a needle is inserted directly into a vein. A solution containing the drug may be given as a single dose or by continuous infusion. With infusion, the solution moves by gravity (from a collapsible plastic bag) or, more commonly, by an infusion pump through thin, flexible tubing into a tube (catheter) inserted into a vein, usually in the forearm.

[0145] In embodiments, the pharmaceutical compositions provided herein are administered by infusion. The infusion may occur over a period of time. For example, the infusion may involve administration of a pharmaceutical agent over a period of about 5 minutes to about 10 hours. The infusion may occur over a period of about 5 minutes, 10 minutes, 20 minutes, 30 minutes, 40 minutes, 50 minutes, 1 hour, 1.5 hours, 2 hours, 2.5 hours, 3 hours, 3.5 hours, 4 hours, 4.5 hours, 5 hours, 6 hours, 7 hours, 8 hours, 9 hours, or up to about 10 hours. In embodiments, intravenous administration is used to deliver precise doses to the entire body in a rapid and well-controlled manner. When administered by subcutaneous or intramuscular injection, irritating solutions are used that can cause pain and tissue damage. When administered intravenously, drugs are delivered immediately to the bloodstream and tend to take effect more quickly than when administered by other routes. As a result, medical professionals closely monitor people who receive intravenous injections for signs that the drug is working or causing unwanted side effects. Additionally, the effects of drugs administered via this route tend to last for a shorter period of time. Therefore, to maintain consistent efficacy, some drugs must be administered via continuous infusion. In some embodiments, infusion reactions may occur, including headache, nausea, drowsiness, difficulty breathing, fever, muscle pain, rash, or other symptoms. Potential risks associated with the administration of anti-ILT7 antibodies or antigen-binding fragments thereof include infection, redness, swelling, pain, and induration at the administration site. Prior to each IV infusion, subjects may receive prophylaxis with IV methylprednisolone, oral diphenhydramine, and oral acetaminophen, or equivalents, to reduce the risk or severity of potential reactions.

[0146] In some embodiments, the drug is administered intrathecally. In the intrathecal route, a needle is inserted between two vertebrae in the lower spine and into the space around the spinal cord. The drug is then injected into the spinal canal. A small amount of local anesthetic is often used to anesthetize the injection site. This route is used when the drug needs to act quickly or locally on the brain, spinal cord, or the tissue layers covering them (meninges), for example, to treat infections in these structures.

[0147] Medications administered by inhalation through the mouth can be atomized into smaller droplets than those administered via the nasal route, allowing the medication to pass through the trachea (bronchi) and enter the lungs. How deeply the droplets penetrate the lungs depends on their size. Smaller droplets penetrate deeper, which increases the amount of medication absorbed. Inside the lungs, they are absorbed into the bloodstream. Medications applied to the skin are typically used for their local effects and are therefore most commonly used to treat superficial skin disorders such as psoriasis, eczema, skin infections (viral, bacterial, and fungal), itching, and dry skin. The medication is mixed with an inactive substance. Depending on the consistency of the inactive substance, the formulation can be an ointment, cream, lotion, solution, powder, or gel.

[0148] In some embodiments, the treatment regimen comprising the pharmaceutical composition can be administered according to the subject's body weight. For subjects who are determined to be obese (BMI>35), it may be necessary to use their actual body weight. In some embodiments, the dosage can be calculated using body surface area.

[0149] In some embodiments, the pharmaceutical composition can be administered by any route, either alone or in combination with a pharmaceutically acceptable carrier or excipient, and such administration can be carried out in both single and multiple doses. More specifically, the pharmaceutical composition can be combined with various pharmaceutically acceptable inert carriers in the form of tablets, capsules, lozenges, troches, hand candies, powders, sprays, aqueous suspensions, injectable solutions, elixirs, syrups, and the like. Such carriers include solid diluents or fillers, sterile aqueous media, and various non-toxic organic solvents. Furthermore, the pharmaceutical formulations can be suitably sweetened and / or flavored with various agents of the type commonly used for such purposes. Exemplary carriers and excipients can include dextrose, sodium chloride (NaCl), sucrose, lactose, cellulose, xylitol, sorbitol, maltitol, gelatin, PEG, PVP, D-histidine / D-histidine hydrochloride, L-histidine / L-histidine hydrochloride, trehalose dihydrate, polysorbate 80, and any combination thereof. In embodiments, the excipient comprises L-histidine / L-histidine hydrochloride, sucrose, and polysorbate 80.

[0150] Numbered Embodiments Notwithstanding the appended claims, the following numbered embodiments also form part of this disclosure.

[0151] Embodiment 1. A method of treating dermatomyositis, the method comprising administering to a subject in need of treatment for dermatomyositis an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof, wherein the ILT7 antibody or antigen-binding fragment thereof comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, the effective amount is about 300 mg, and the antibody is administered once every four weeks.

[0152] Embodiment 2. The method of embodiment 1, wherein the dermatomyositis includes well-defined myositis.

[0153] Embodiment 3. The method of embodiment 1, wherein the dermatomyositis includes possible myositis.

[0154] Embodiment 4. A method for treating anti-synthetase inflammatory myositis (ASIM), the method comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of treatment for ASIM, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, the effective amount is about 300 mg, and the antibody is administered once every four weeks.

[0155] Embodiment 5. The method of embodiment 4, wherein ASIM includes well-defined myositis.

[0156] Embodiment 6 The method of embodiment 4, wherein the ASIM includes possible myositis.

[0157] Embodiment 7. A method of treating a disease, the method comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of disease treatment, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, the effective amount comprising a dose of about 300 mg, and the administering is completed once every four weeks.

[0158] Embodiment 8. A method of treating a disease, the method comprising administering an effective amount of an anti-Immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of disease treatment, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and wherein the administration is effective to alleviate the disease as compared to (a) the disease in an otherwise comparable subject lacking the administration, or (b) a baseline measure of the disease in the subject in need of disease treatment.

[0159] Embodiment 9. The method of any one of embodiments 1-8, wherein the administering continues for at least about 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, or up to about 15 administrations.

[0160] Embodiment 10. The method of any one of embodiments 8-9, wherein the administering is (b) effective in alleviating disease in a subject in need of treatment for the disease, as compared to a baseline measure of the disease, wherein the alleviation is measured by detecting a decreased total improvement score in the subject.

[0161] Embodiment 11. The method of any one of embodiments 8-9, wherein the administering is effective in alleviating disease in a subject in need of treatment for the disease, as compared to a baseline measure of the disease, wherein the alleviation is measured by detecting a reduced level of a symptom of the disease in the subject.

[0162] Embodiment 12. The method of any one of embodiments 10-11, wherein the measurements are completed after 6 and / or 12 administrations.

[0163] Embodiment 13. The method of any one of embodiments 10 to 12, wherein the total improvement score is 10 or more, 20 or more, 30 or more, 40 or more, or 50 or more.

[0164] Embodiment 14. The method of embodiment 13, wherein the total improvement score is 20 or greater.

[0165] Embodiment 15. The method of embodiment 13, wherein the total improvement score is 40 or greater.

[0166] Embodiment 16. The method of any one of embodiments 11-15, wherein the level of symptom reduction comprises a decrease in a score on a survey selected from the group consisting of the Dermatomyositis Disease Area and Severity Index (CDASI), the Myositis Disease Activity Assessment Tool (MDAAT), the Myositis Damage Index (MDI), and combinations thereof, compared to the baseline.

[0167] Embodiment 17. The method of any one of embodiments 11-15, wherein the level of symptom reduction comprises an increase in pulmonary compliance as measured by an increase in forced vital capacity (FVC) compared to baseline.

[0168] Embodiment 18. The method of any one of embodiments 8-9, wherein the administering is (b) effective in reducing disease in a subject in need of treatment for the disease compared to a baseline measure of the disease, where the reduction is measured by a reduction in corticosteroid use, and the measurement is completed after six doses.

[0169] Embodiment 19. The method of embodiment 18, wherein the corticosteroid comprises prednisone.

[0170] Embodiment 20. The method of any one of embodiments 18-19, wherein the reduction comprises a decrease in frequency of use of at least about 10%, 15%, 20%, 25%, 30%, or 40%.

[0171] Embodiment 21. The method of any one of embodiments 18-19, wherein the reduction is from a dose of about 10 mg / day or more to a dose of 7.5 mg / day.

[0172] Embodiment 22. The method of any one of embodiments 1 to 21, wherein the ILT7 antibody comprises a heavy chain variable region (VH) and a light chain variable region (VL), wherein the VH and VL regions comprise amino acid sequences that are at least 80% identical to SEQ ID NO:2 and SEQ ID NO:7, respectively.

[0173] Embodiment 23. The method of any one of embodiments 1 to 22, wherein the ILT7 antibody comprises a heavy chain variable region (VH) and a light chain variable region (VL), wherein the VH and VL regions comprise the amino acid sequences of SEQ ID NO: 2 and SEQ ID NO: 7, respectively.

[0174] Embodiment 24. The method of any one of embodiments 1 to 23, wherein the ILT7 antibody is a monoclonal antibody.

[0175] Embodiment 25. The method of any one of embodiments 1 to 24, wherein the ILT7 antibody is nonfucosylated.

[0176] Embodiment 26. The method of any one of embodiments 4 to 25, wherein the required subject sample is positive for one or more ASIM-associated antibodies.

[0177] Embodiment 27. The method of embodiment 26, wherein the one or more ASIM-related antibodies are selected from the group consisting of anti-Jo-1, anti-PL-12, anti-PL-7, anti-KS, anti-EJ, anti-OJ, anti-ZO, and anti-YRS(HA).

[0178] Embodiment 28. The method of any one of embodiments 1 to 27, wherein the subject in need thereof has a dermatomyositis-associated rash.

[0179] Embodiment 29. The method of any one of embodiments 1 to 28, wherein the disease is caused by plasmacytoid dendritic cells.

[0180] Embodiment 30. The method of any one of embodiments 1 to 29, wherein the administration is effective in reducing the level of type I interferon in the subject compared to the subject's baseline level.

[0181] Embodiment 31. The method of embodiment 30, wherein the level is reduced by at least about 1-fold, 5-fold, 10-fold, 25-fold, 50-fold, 75-fold, 100-fold, 150-fold, 250-fold.

[0182] Embodiment 32. The method of any one of embodiments 7 to 31, wherein the administering is effective in eliminating the disease for at least about 3 months, 6 months, 1 year, or 2 years.

[0183] Embodiment 33. The method of any one of embodiments 1 to 32, wherein administration is subcutaneous.

[0184] Embodiment 34. A method for treating dermatomyositis, the method comprising subcutaneously administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of treatment for dermatomyositis, wherein the ILT7 antibody or antigen-binding fragment thereof comprises complementarity-determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and the effective amount is about 300 mg.

[0185] Embodiment 35. A method for treating anti-synthetase inflammatory myositis (ASIM), the method comprising subcutaneously administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of treatment for ASIM, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and the effective amount is about 300 mg. [Example]

[0186] Example 1 - A Phase 2, randomized, double-blind, placebo-controlled, efficacy and safety study of subcutaneous injection of daxudilimab in adult participants with well-controlled dermatomyositis or antisynthetase inflammatory myositis. Disclosed herein is a phase 2, randomized, double-blind, placebo-controlled, efficacy and safety study of subcutaneous injection of daxudilimab in adult patients with well-controlled dermatomyositis or antisynthetase inflammatory myositis.

[0187] Objectives and evaluation items The study objectives and endpoints are presented in Table 2 and were the same for DM and ASIM, ensuring that the analyses of the two populations were independent.

[0188] [Table 2-1]

[0189] [Table 2-2]

[0190] [Table 2-3]

[0191] Study design This study is a phase 2, randomized, double-blind, placebo-controlled, proof-of-concept study to investigate the efficacy and safety of daxudilimab in participants aged 18 to 75 years with dermatomyositis (DM) or antisynthetase inflammatory myositis (ASIM).

[0192] The study will enroll two populations of participants with idiopathic inflammatory myositis (Figure 1): Population 1 or DM: Participants with definite or probable myositis according to the American College of Rheumatology / European League Against Rheumatism 2017 (ACR / EULAR 2017) (Lundberg et al., 2019) criteria and DM rash. Population 2 or ASIM: Participants with ASIM, with definite or probable myositis according to the ACR / EULAR 2017 criteria and positive ASIM-associated antibodies.

[0193] Up to 96 participants will be randomized 1:1 by population (24 participants per group) to receive the investigational product (IP; daxudilimab 300 mg or placebo) by subcutaneous (SC) injection once every four weeks (Q4W). Group 1: DM placebo Group 2: DM daxuzilimab 300 mg Group 3: ASIM placebo Group 4: ASIM daxudilimab 300mg

[0194] Randomization can occur up to 28 days after successful screening and will be stratified by Manual Muscle Testing 8 (MMT8) score (<120 vs. >120) at baseline (Day 1). The estimated total study duration, from screening to the end of the safety follow-up (SFU) period, is up to 60 weeks with clinic visits Q4W (Table 3): 32. Approximately 4-week screening period (Day -28 to Day -1) 33. Treatment period from Day 1 to Week 48 Randomization on Day -1 (double-blind) IP administration from Day 1 through Week 44 -Efficacy and safety survey up to 48 weeks Q4W -Consists of a randomized controlled period (RCP) and an open-label extension (OLE) period: a. Randomized controlled period (double-blind) from Day 1 to Week 24 The primary efficacy endpoint will be assessed at 24 weeks, after which time participants in the placebo groups (Groups 1 and 3) will switch to receive 300 mg of daxuzilimab SC Q4W for an additional 24 weeks, and participants initially randomized to daxuzilimab 300 mg (Groups 2 and 4) will continue to receive 300 mg of daxuzilimab SC Q4W. b. Open-label extension from 24 to 48 weeks All participants will begin active treatment from week 24, but will remain blinded to previous treatment (prior to week 24).

[0195] All IP doses will be administered in the clinic by an unblinded pharmacist or nurse, and participants may be observed for at least 60 minutes after the first and second doses and for 20 minutes after all subsequent doses. To maintain blinding to previous treatment, all participants may be observed for at least 60 minutes after IP doses at 24 and 28 weeks.

[0196] Efficacy studies can include a series of scores, scales, and other measurements to assess disease activity, damage, and improvement.

[0197] Patient-reported outcomes can be used to document participants' well-being and quality of life during the study.

[0198] Blood samples from all participants and optional muscle and / or skin biopsies from consenting participants can be collected to characterize the pharmacokinetics (PK), pharmacodynamics (PD), and immunogenicity of daxudilimab.

[0199] Study investigations scheduled for Day 1 can be performed prior to IP administration; patient-reported outcomes will be performed first, followed by physician investigations, vital sign measurements, and laboratory investigations.

[0200] In the event of significant study continuity issues (e.g., caused by a pandemic), alternative methods for participant visits, surveys, medication distribution, and monitoring can be implemented. The study may be conducted entirely on an outpatient basis.

[0201] Definition of end of study End of study was defined as the last visit of the last participant during the study or the last scheduled procedure (56 weeks) as shown in the Schedule of Assessments (SoA; Table 3) for the last participant during the entire study.

[0202] A participant was considered to have completed the study if he or she completed the entire study period, including the last visit or last scheduled procedure (week 56) shown in Table 3.

[0203] [Table 3-1]

[0204] [Table 3-2]

[0205] [Table 3-3]

[0206] [Table 3-4]

[0207] Study population Eligible participants for this study may have DM or ASIM.

[0208] Inclusion criteria Participants who meet the following criteria will be eligible to be included in this study:

[0209] Age and gender 1. Adult male or female, aged 18-75 years at the time of signing the informed consent form (ICF).

[0210] Participant type and disease characteristics 2. Diagnosis of definite or probable myositis according to the ACR / EULAR 2017 criteria: A total aggregate score of 5.5 or greater, excluding muscle biopsy A total aggregate score of 6.7 or greater, including muscle biopsy If muscle biopsy is not available, etiological skin rash (heliotrope rash, Gottron papules, and / or Gottron sign) is required NOTE: Local muscle biopsy reports are used in the ACR / EURLAR 2017 criteria to measure participant eligibility. Submission of a historical biopsy sample (archived tissue block, physical slide, and / or digital pathology slide) is required to confirm randomized participants. a. Group 1: DM A diagnosis of DM due to a current or past DM rash, or b. Group 2: ASIM Anti-Jo-1 antibodies must be positive during screening by central laboratory testing, or · One of the following antibodies must be positive by historical testing: anti-PL-12, anti-PL-7, anti-KS, anti-EJ, anti-OJ, anti-ZO, anti-YRS (HA). Note: Participants who have a DM rash and test positive for anti-Jo-1 antibodies during screening will be considered part of population 2. Participants with a history of anti-Jo-1 who test negative by central laboratory anti-Jo-1 during screening will not qualify. 3. Currently active myositis with all of the following (a, b, and c) during screening: a. MMT8 score less than 142 b. At least two other Core Set Measures (CSM) from the following list: PtGDA of 2 cm or more on a 10 cm visual analogue scale (VAS) Physician-Determined Total Disease Activity (PhGDA) of 2 cm or more on a 10-cm VAS Extramuscular activity of 2cm or more on a 10cm VAS At least one muscle enzyme at 1.5 times the upper limit of normal (ULN) HAQ-DI of 0.5 or higher c. Myositis Damage Index (MDI) total muscle damage score of 5 or less on a 10cm VAS 4. Participants should be receiving stable standard of care therapy if tolerated (a); if they are unable to tolerate this therapy or if they fail standard of care, medication must be discontinued (b): a. Patients receiving corticosteroid treatment (maximum 20 mg prednisone or equivalent / day) and / or up to two non-excluded immunosuppressants on stable therapy (Table 6) for at least 4 weeks prior to randomization, or b. Patients who had previously failed to respond or previously intolerant to corticosteroids and at least one additional immunosuppressant, and in whom the steroids / immunosuppressants had been discontinued (Table 6). 5. Participants should be willing to taper their corticosteroid dose per protocol when stable or improving.

[0211] Gender and contraceptive / barrier requirements 6. Female participants are eligible to participate if they are not pregnant or lactating and one of the following conditions applies: a. Are a woman of non-childbearing potential (WONCBP), or b. Women of childbearing potential (WOCBP) AND: i. A negative serum pregnancy test at screening and a negative urine pregnancy test on Day 1. ii. Agree to use highly effective contraception from at least 4 weeks prior to Day 1 until at least 3 months after the last dose of IP, or until the end of the study, whichever is longer. iii. The investigator will be responsible for reviewing medical history, menstrual history, and recent sexual activity to reduce the risk of including women with early undetected pregnancy. 7. Male participants are eligible to participate if they agree to the following during the study and for at least 3 months after the last dose of IP: a. Refrain from donating sperm from Day 1 until at least 3 months after the last dose of IP. Plus one of the following: b. Agree to abstain (long-term and sustained) from heterosexual intercourse and maintain abstinence as their preferred and usual lifestyle; or c. If he has sexual intercourse with a woman of childbearing potential who is not currently pregnant, he agrees to use a male condom and an additional highly effective method of contraception from Day 1 through at least 3 months after the last dose of IP. If the male participant's female partner uses a hormonal contraceptive method, this method must be used by the female partner from at least 4 weeks prior to Day 1 through at least 3 months after the last dose of IP, or until the end of the study, whichever is longer.

[0212] Exclusion criteria Participants will be excluded from the study if any of the following criteria apply:

[0213] General exclusion criteria 1. Participation in another clinical study with an investigational drug within 4 weeks or 5 published half-lives prior to randomization, whichever is longer.

[0214] medical conditions 2. Any condition that, in the opinion of the investigator or sponsor, would interfere with the evaluation of the IP or with the safety of the participant or the interpretation of the study results. 3.Weight greater than 160 kg (352 lbs) at screening. 4. History of allergy, hypersensitivity reaction, or anaphylaxis to any component of the IP or to previous mAb or human Ig therapy. 5. Women who are breastfeeding or pregnant, or who intend to become pregnant anytime between signing the ICF and 6 months after receiving the last dose of IP. 6. A history of drug abuse or alcoholism that, in the investigator's opinion, may affect the participant's safety or compliance at visits or interfere with other study investigations. 7. Major surgery within 8 weeks prior to screening or elective surgery planned within 48 weeks from screening. 8. Having undergone spontaneous or induced abortion (stillbirth or live birth) or being pregnant within 4 weeks prior to screening and randomization. 9. History of clinically significant cardiac disease within 6 months prior to randomization, including unstable angina, myocardial infarction, or congestive heart failure; arrhythmia requiring aggressive therapy, excluding clinically insignificant premature beats or minor cardiac conduction abnormalities; or the presence of clinically significant abnormalities on ECG that, in the investigator's opinion, increase the risk of study participation. 10. Have a history of cancer within the past 5 years, except for the following: In situ cervical cancer that has been apparently successfully treated with curative therapy more than 12 months prior to screening, or Basal or squamous cell carcinoma of the skin treated with curative therapy. 11. Any underlying medical condition that, in the investigator's opinion, significantly predisposes the participant to infection. 12. Participant who receives more than 50 mL of blood or plasma within 30 days of screening (during a clinical study or at the time of blood bank donation), or more than 499 mL of blood or plasma within 56 days of screening, or who plans to receive blood or plasma during study participation or up to 6 months after the last dose of IP, whichever is longer. 13. Receiving blood, packed red blood cell, or platelet transfusions, or plasmapheresis, or plasma exchange within 8 weeks prior to randomization and throughout study participation.

[0215] Laboratory Standards 14. History of primary immunodeficiency or underlying disease, such as known human immunodeficiency virus (HIV) infection or a positive central laboratory result for HIV infection. 15. At screening, central laboratory testing results in one of the following (may be repeated once within the same screening period to confirm results prior to randomization): Total bilirubin above 1.5 × ULN (excluding those due to Gilbert's syndrome) Less than 1500 / μL (or 1.5×10 9 Neutrophil count (less than / L) Less than 135,000 / μL (or 135 x 10 9 / L) Hemoglobin less than 10g / dL (or less than 100g / L) Less than 800 / μL (or 0.8×10 9 Total lymphocyte count (less than / L)

[0216] Infection criteria 16. Confirmed positive test for Hepatitis B virus serology defined as: Hepatitis B surface antigen, or Hepatitis B core antibody 17. Participants with active tuberculosis (TB) or a positive IFN-γ release assay (IGRA) or indeterminate IGRA test result at screening, unless they have a documented history of appropriate treatment for active or latent TB according to local guidelines, may repeat the test, but they will be excluded if the repeated test is also indeterminate. 18. Any serious herpesvirus family infection (including Epstein-Barr virus, cytomegalovirus [CMV]) at any time prior to randomization, including but not limited to disseminated herpes, herpes encephalitis, recent recurrent herpes zoster (defined as two episodes within the past two years), or ophthalmic herpes. 19. Any herpes zoster, CMV, or Epstein-Barr virus infection that has not fully resolved in the 12 weeks prior to randomization 20. Any of the following within 30 days prior to signing the ICF and through randomization: Clinically significant active infection, including ongoing and chronic infections requiring antibiotic or antiviral medication, in the opinion of the investigator (chronic nail infections are permitted). Any infection requiring hospitalization or treatment with intravenous anti-infective agents. Participants with a documented positive SARS-CoV-2 test may be rescreened at least two weeks after the positive test if the participant is asymptomatic and at least three weeks for symptomatic COVID-19 illness. 21. Opportunistic infection requiring hospitalization or parenteral antimicrobial treatment within 2 years prior to randomization. 22. No evidence of any acute illness or clinically significant active infection on Day 1. 23. Participants who have, or who, in the judgment of the investigator, may be at higher risk for COVID-19 or other serious infectious diseases, or whose complications exist, should not be randomized.

[0217] Disease-related criteria 24. Significant organ system impairment or myositis damage (total muscle damage score greater than 5 on the 10 cm VAS scale on the MDI) that poses a risk during the study or interferes with the investigation. 25. Diagnosis of immune-mediated necrotizing myopathy (IMNM) (positive anti-HMGR, anti-SRP, or antibody negative), inclusion body myositis (IBM) (including positive anti-cN1A), or drug-induced myositis. 26. Current musculoskeletal, joint, or inflammatory disease, including significant joint contractures or calcification, which, in the investigator's opinion, may interfere with muscle strength studies and confuse disease activity studies. 27. Wheelchair participant 28. Current inflammatory skin disease, other than DM or ASIM, that, in the investigator's opinion, may interfere with the inflammatory skin survey or confuse the disease activity survey. 29. Severe interstitial lung disease with respiratory symptoms limiting the participant's function or progressive pulmonary fibrosis. 30. Myositis overlapping with another connective tissue disease, which prevents accurate assessment of treatment response (e.g., difficulty assessing muscle strength in scleroderma patients with associated myositis).

[0218] Previous treatments / combination therapies 31. Use of a Janus kinase inhibitor within 4 weeks prior to randomization. 32. Use of cyclophosphamide within 3 months prior to randomization. 33. Use of IVIG within 4 weeks prior to randomization. 34. Previous treatment with any biologic B-cell depleting therapy (e.g., rituximab, ocrelizumab, or ofatumumab) or other B-cell targeted therapy (e.g., belimumab) within 3 months prior to randomization. B cells must be detectable after treatment with the therapy prior to randomization. 35. Prior treatment with anti-BDCA2 [BIIB059] within 6 months prior to randomization. 36. Participants who have received prior treatment with daxuzilimab. 37. Taking any biologic or antiproliferative medication when the final dose was received: Within 4 weeks prior to randomization, or Within 5 drug-specific half-life elimination periods (if longer than 4 weeks) before randomization. 38. Exposure to any experimental drug for 30 days, 5 half-lives of the drug, or twice the duration of the drug's biological effect, whichever is longer, between randomization and the first study visit. 39. Received a live attenuated vaccine within 4 weeks prior to randomization.

[0219] Lifestyle Considerations There are no dietary, caffeine, alcohol, or tobacco restrictions in this study. Participants are only asked to refrain from strenuous exercise for 24-48 hours before each blood draw for clinical laboratory tests. Participants may participate in light resting activities (e.g., watching television, reading) during study visits.

[0220] Investigational Products and Combination Therapies All investigational products are prescription drug products (i.e., daxudilimab and placebo) intended for administration to study participants during the conduct of the study.

[0221] Administered IP IP daxudilimab can be supplied as a 2R vial with a nominal 1 mL of 100 mg / mL daxudilimab containing 20 mM L-histidine / L-histidine HCl, 240 mM sucrose, 0.02% (w / v) polysorbate 80, pH 6.0.

[0222] The placebo can be supplied as a 2R vial with a nominal 1 mL of 20 mM L-histidine / L-histidine HCl, 240 mM sucrose, 0.02% (w / v) polysorbate 80, pH 6.0.

[0223] Both IPs can be administered by SC injection Q4W from Day 1 through Week 24. After Week 24 through Week 44, daxudilimab alone can be administered to all participants. To maintain the same number and volume of injections across all groups, the required dose can be administered as two 1.5 mL injections.

[0224] Further details regarding IP can be found in Table 4.

[0225] [Table 4]

[0226] [Table 5]

[0227] Blinding Participants will be randomly assigned in a 1:1 ratio to receive daxudilimab 300 mg or placebo on Day 1 (see Table 5 for study arms). Up to 24 weeks, the study can be double-blind, meaning that participants, investigators, and site personnel do not know which IP the participant is receiving. At 24 weeks, the OLE period of the study begins. Participants in the placebo group (Groups 1 and 3) are switched to 300 mg of daxudilimab. Participants initially randomized to the daxudilimab 300 mg group (Groups 2 and 4) remain on that same dose for the remainder of the study. -All participants will receive active treatment from week 24, but will remain blinded to previous treatment (prior to week 24).

[0228] Permitted Treatments Participants may be on permitted standard of care medications (Table 6) if stable prior to screening: Azathioprine, tacrolimus, cyclosporine, plaquenil, and mycophenolate mofetil must be on stable doses for at least 4 weeks prior to screening and throughout the study. Methotrexate (oral or SC) must be at a stable dose and route of administration for at least 4 weeks prior to screening and throughout the study. Corticosteroids must be on a stable dose of up to 20 mg / day prednisone equivalent for at least 4 weeks prior to screening. If the participant is stable or improving, the investigator will tapere the prednisone between weeks 4 and 20 and during OLE (Table 9).

[0229] If a participant does not respond to, tolerate, and / or decides to discontinue medication, medication may be discontinued following a washout period at the Day 1 visit (Table 6), prior to the first dose of IP, and until the final study visit.

[0230] [Table 6]

[0231] Prohibited treatments Participants who initiate prohibited medications or new therapies (except those permitted as concomitant medications) shown to be effective in treating DM or ASIM during the study may be removed from the IP.

[0232] Participants who initiate prohibited medications or treatments during the study for other reasons may be removed from study treatment if this is anticipated to affect the participant's efficacy or safety.

[0233] Table 7 lists prohibited medications that cannot be used before the first dose of IP at the Day 1 visit and during the defined washout period until the final study visit.

[0234] [Table 7]

[0235] Salvage therapy Rescue therapy is any new background therapy or an increase in dose above the Day 1 baseline of any permitted therapy given to the participant.

[0236] For some rescue medications, IP may continue after discussion and approval by the medical monitor. However, if a participant receives a prohibited medication as rescue, the participant may be discontinued from IP but remain in the study for safety investigations.

[0237] Rescue medication for worsening DM or ASIM may be administered at the investigator's discretion as clinically indicated.

[0238] Rescue medication use is permitted for clinical deterioration but may be delayed, if possible, until week 24. Medical monitors should weigh the urgency of providing rescue medication against the proximity of the primary endpoint (week 24).

[0239] Participants receiving salvage therapy may be considered treatment-ineligible for subsequent endpoints.

[0240] The date and time of rescue medication administration, as well as the name and dosing regimen of the rescue medication, can be recorded.

[0241] Research Survey and Procedures Study procedures and their timing are summarized in Table 3. Compliance with study design requirements, including those specified in Table 3, may be required for the conduct of the study. · Complete and review all screening assessments to ensure potential patients meet all eligibility criteria. Procedures performed as part of the participant's routine clinical management and obtained prior to signing of the ICF may be used for screening or baseline purposes, provided the procedures meet the criteria specified in the protocol and are performed within the timeframes defined in Table 3. In the event of significant study continuity issues (e.g., caused by a pandemic), alternative methods for participant visits, surveys, medication distribution, and monitoring can be implemented. · Safety, laboratory, and / or specimen results that could unblind the study cannot be reported to the investigational institution or other blinded personnel. Repeat or unscheduled samples may be taken for safety reasons or due to technical problems with the sample.

[0242] Efficacy evaluation The planned time points for all efficacy studies are shown in Table 3.

[0243] Efficacy can be investigated by: 1. Total Improvement Score (TIS) based on 6 CSMs: a. Health Assessment Questionnaire Disability Index (HAQ-DI) b. Patient Total Disease Activity (PtGDA) c. Physician's Global Disease Activity (PhGDA) (part of the Myositis Disease Activity Assessment Tool [MDAAT]) d. Bilateral Manual Muscle Testing 8 (MMT8) e. Laboratory Muscle Enzymes f. Extramuscular total survey (part of MDAAT) 2. Total MDAAT 3. Dermatomyositis Disease Area and Severity Index (CDASI) 4. Steroid tapering 5.Myositis Damage Index (MDI) 6.Myositis Functional Index 3 (FI3) (optional) 7. Forced vital capacity (FVC) 8. Handheld Dynamometry (Optional) 9.28 joints 10. Actigraphy test (optional) 11. Magnetic Resonance Imaging (MRI) Scan

[0244] TIS based on six CSMs The TIS is derived from standardized clinical response criteria recommended for use as a primary endpoint in drugs for the treatment of myositis. The criteria use six CSMs, each with an absolute percentage (%) change combined with variable weighting to yield a TIS on a scale of 0 to 100. Various thresholds of improvement can be set for minimal, moderate, and major response. The TIS can be used as a continuous outcome measure or as a categorical outcome of improvement (minimal, moderate, or major improvement); it is therefore a hybrid measure. Improvement category thresholds for minimal, moderate, and major improvement have been developed and validated for DM and PM. (See online calculator in MRC 2016.)

[0245] HAQ-DI The HAQ-DI examines physical function. It includes eight sections: dressing and grooming, getting up, eating, walking, hygiene, reaching, grasping, and general daily activities. For each of these categories, participants report the amount of difficulty they have in performing two or three specific activities. Scoring within each section ranges from 0 (no difficulty) to 3 (unable to do). The time frame for the disability questions is the past week. The disability index is designed to examine participants' usual abilities using conventional equipment.

[0246] PtGDA The PtGDA measures a patient-wide assessment of disease activity at the time of the survey, using a 10 cm VAS ranging from "no evidence of disease activity" to "very active or severe disease activity."

[0247] PhGDA (part of MDAAT) The PhGDA is a physician's judgment of overall disease activity (extramuscular organ systems and muscle disease activity) based on all clinical laboratory investigations. It uses a 10-cm VAS ranging from "no evidence of disease activity" to "extreme or maximal disease activity" and a 5-point Likert scale ranging from 0 (no activity) to 4 (very severe activity).

[0248] Participants may be considered to have a complete response if, during the study, they have no evidence of disease activity despite receiving treatment (i.e., PhGDA VAS=0).

[0249] Bilateral MMT8 The bilateral MMT8 is a series of eight specified muscles tested bilaterally (potential score 0-140). Because axial (neck flexor) testing is included, the potential score for the MMT8 is 150.

[0250] Laboratory Muscle Enzymes Creatine kinase, transaminases (alanine transaminase [ALT] and aspartate transaminase [AST]), lactate dehydrogenase, and aldolase levels will be measured to assess the effect of daxudilimab on muscle-related enzymes.

[0251] Extramuscular total survey (part of MDAAT) The extramuscular gross survey provides an overall assessment of disease activity in all extramuscular systems (excluding muscle disease activity) using a 10 cm VAS ranging from "no evidence of disease activity" to "extreme or maximal disease activity."

[0252] Total MDAAT The MDAAT (V 2.0) measures the degree of disease activity in extramuscular organ systems and muscles by combining a 10 cm VAS, ranging from "no evidence of disease activity" to "extreme or maximal disease activity," and an activity index, ranging from 0 ("absent in the last 4 weeks") to 4 ("new in the last 4 weeks").

[0253] CDASI The CDASI (V 2.0) is an outcome measure used to assess the severity of cutaneous DM and detect improvement in disease activity. The scale rates disease improvement in 15 different body regions using three activity (erythema, scale, erosion / ulceration) and two damage (poikiloderma, calcinosis) scales. The presence and severity of Gottron papules on the hands, periungual changes, and alopecia are also captured. Activity scores range from 0 to 100, and damage scores range from 0 to 32. Higher scores indicate greater disease severity.

[0254] Steroid tapering For participants who are taking corticosteroids (e.g., prednisone or methylprednisone) and are stable or improving, investigators will taper the dose by 2.5 mg / day (prednisone equivalent) every 4 weeks from Weeks 4 to 20 (Table 8). Note that corticosteroid doses will not be allowed to change between Weeks 20 and 24 (Figure 1).

[0255] [Table 8]

[0256] For participants who remain stable or improve during OLE, investigators must continue to tapering participants off a clinically tolerated corticosteroid (e.g., prednisone or methylprednisone). The recommended tapering is 2.5 mg / day every 4 weeks to 5 mg / day, then 1 mg / day every 2 weeks until discontinuation (Table 9).

[0257] [Table 9]

[0258] If symptoms worsen, participants can maintain their current dose or go back to their previous dose.

[0259] Criteria for deterioration and maintaining steroid tapering If the participant is not stable or improving and the deterioration criteria (see below) are met, steroid tapering may be maintained.

[0260] Steroid tapering can be maintained if PhGDA worsens globally from baseline by 2 cm or more on a 10 cm VAS and at least one of the following occurs: -MMT8 score worsens by 20% or more from baseline, or MDAAT VAS: 2 cm or more from baseline, deterioration of total extramuscular activity, or - 30% or more worsening of any three CSMs from baseline

[0261] Criteria for confirming deterioration and defining treatment failure and offering alternative treatments or steroid bursts Participants may be offered an alternative treatment or steroid tapering regimen if, over two consecutive visits, PhGDA worsens globally by 2 cm or more from baseline on a 10 cm VAS and at least one of the following occurs (over the same two consecutive visits): - A 20% or greater deterioration from baseline in MMT8 score on two consecutive visits, or - A worsening of total extramuscular activity by 2 cm or more from baseline on the MDAAT VAS at two consecutive visits, or - 30% or greater deterioration from baseline in any three CSMs at two consecutive visits

[0262] MDI The MDI assesses the degree of disease damage to all organ systems and consists of a series of organ-specific questions regarding the presence or absence of given signs or symptoms or problems to measure the degree of damage, and an overall rating of disease damage for each system using a 10 cm VAS to measure the severity of damage, from "no evidence of disease damage" to "extreme or maximal disease damage."

[0263] Myositis FI3 The Myositis FI3 is a functional outcome designed to assess muscle endurance and function of frequently affected muscle groups. Each muscle group is scored as the number of repetitions performed correctly, with a maximum of 60 or 120 repetitions depending on the muscle group.

[0264] FVC Pulmonary function testing at a local pulmonary function laboratory can be performed to measure participants' FVC.

[0265] Handheld Dynamometry Handheld dynamometry can be used to assess muscle strength. This procedure has shown strong reliability, construct validity, and responsiveness in patients with myositis (Baschung et al., 2018), particularly for assessing the strength of single muscle groups (Saygin et al., 2021).

[0266] 28 joints Painful, swollen, and tender joint counts are based on the left and right elbows, wrists, metacarpal (MCP) 1, MCP2, MCP3, MCP4, MCP5, proximal interphalangeal (PIP) 1, PIP2, PIP3, PIP4, and PIP5 joints of the upper extremities, and left and right knees of the lower extremities (Scott et al., 2014). At the start of the joint count (before the tenderness and swelling survey), participants may be asked whether they have experienced or are experiencing pain in any of the 28 joints within the past 30 days. Tenderness (by palpation of the joint) and swelling of any of the 28 joints are then assessed individually. Joints that have received intra-articular injections within the past 4 weeks cannot be assessed in this survey.

[0267] The joint count survey can include questions about limitations in range of motion and the impact of joint symptoms on basic and functional activities of daily living.

[0268] Actigraphy To measure changes in daily activities, participants may wear an actigraphy device at each visit for one week. If desired, the actigraphy device can be worn more frequently. Participants must bring their actigraphy device to each visit so that data can be downloaded.

[0269] MRI scan For participants who consent to a muscle biopsy, a regional MRI scan can be used to measure the muscle biopsy site.

[0270] Clinical Safety Laboratory Testing · Laboratory testing (Table 10) includes urinalysis, hematology with differential, standard chemistry panel (chemistry includes liver function tests), and a serum pregnancy test at the time of screening for WOCBP. All laboratory tests required by the clinical trial protocol must be performed in accordance with Table 3.

[0271] [Table 10-1]

[0272] [Table 10-2]

[0273] Pregnancy test · Serum β-human chorionic gonadotropin (βhCG) pregnancy test will be performed for all WOCBPs by the central laboratory at the time of screening. · Urine pregnancy tests will be performed by study staff at the clinic and prior to administration of IP, from Day 1 through Week 48, and at the end of study visit (Week 56).

[0274] Pharmacokinetics Approximately 3.5 mL serum samples (whole blood collection tubes) can be collected for measurement of daxudilimab serum concentrations at the time of visit and at the time points specified in Table 3.

[0275] Samples may be collected at further time points during the study if approved and agreed upon between the investigator and the sponsor.

[0276] Samples collected for analysis of daxudilimab serum concentrations can also be used to evaluate safety and efficacy aspects for any concerns that arise during or after the study.

[0277] Intervention concentration information that unblinds the study cannot be reported to survey sites or blinded personnel.

[0278] Pharmacodynamics The planned time points for all PD studies are shown in Table 3.

[0279] While it may not be possible to provide a comprehensive list of biomarkers at the start of a study as new research within the disease may generate or publish new information related to daxuzilimab or new analytical techniques during the course of the study. However, the effect of daxuzilimab on PD biomarkers can be investigated by: Levels of pDCs in blood (pDC flow cytometry) and tissues (e.g., muscle and skin biopsy immunohistochemistry [IHC]) ·Serum IFN-α Blood xenovirus resistance protein A (MxA) Interferon gene signature levels Human peripheral blood mononuclear cells (PBMCs)

[0280] pDC levels in blood and tissues Levels of circulating pDCs can be measured in the blood using multiparameter flow cytometry.

[0281] At the time points listed in Table 3, optional muscle and skin biopsies can be performed to examine whether daxudilimab alters the levels of pDCs, inflammatory cells, and / or other biomarkers in the tissue.

[0282] Skin biopsy Biopsies may be performed at baseline before dosing on Day 1 or during the screening period, but not until other screening procedures have confirmed that the participant is eligible for the study. The site selected for skin biopsy should have an active rash by default. The biopsy may require a 4mm punch biopsy, and it is recommended that the punch biopsy site be closed with a single suture. · Skin biopsies should be repeated at the reference biopsy site from the same anatomic location, avoiding any scar tissue from a previous punch biopsy. The samples are formalin-fixed and paraffin-embedded to allow for IHC and other analyses.

[0283] Muscle biopsy Participants who consent to a muscle biopsy can first undergo an MRI scan at either the first or second visit, followed by a muscle biopsy on the same or the next day. These procedures should be performed prior to dosing. Day 1 visit procedures for these participants may take up to three days to complete. Once collected, these participants can be dosed one day after the biopsy. Day 1 can be counted from the date of the first dose. The anatomical site selected for muscle biopsy should be in an area with active inflammation on MRI. For a biopsy, a sample of muscle tissue can be taken by inserting a biopsy needle into the muscle. If a larger sample is needed, an incision can be made in the skin (open biopsy) and a larger section of muscle can be removed. Muscle biopsies should be repeated from the contralateral muscle to the reference biopsy site.

[0284] Serum IFN-α Serum samples can be collected to investigate changes in levels of cytokines, chemokines, or other proteins (e.g., IFN-α, IL-17, and IFN-γ) that may be related to the pathogenesis of DM or ASIM or the mechanism of action of daxudilimab.

[0285] Blood MxA Blood can be collected to investigate changes in the levels of MxA protein, which may be related to the pathogenesis of DM / ASIM or the mechanism of action of daxudilimab.

[0286] IFN gene signature level Blood can be collected to investigate changes in the levels of IFN gene signatures that may be related to the pathogenesis of DM or ASIM or the mechanism of action of daxudilimab.

[0287] PBMC Human peripheral blood mononuclear cells can be collected to monitor changes in frequency, and optionally gene expression, of immune cell populations in cryopreserved blood leukocytes.

[0288] Machine / Disease Biomarkers Serum can be collected to measure changes in exploratory biomarkers of disease activity, changes in biomarkers related to the mechanism of action of daxudilimab, and changes in biomarkers resulting from daxudilimab administration.

[0289] Specific proteins that can be investigated include, but are not limited to, IFN-α and MxA to measure the effect of daxudilimab on interferon response. These analyses can be performed using both multiplexed assays for panels of biomarkers and individual immunoassays for specific markers of interest.

[0290] It should be noted that not all assays may be performed on all participants at each time point. The decision to perform assays can be made based on considerations including statistical and scientific design, available technology, and study outcomes.

[0291] Metabolomics / Proteomics Whole blood samples can be collected and RNA isolated (Whole Blood Transcriptomics, Table 3) to investigate changes in gene expression or gene pathways associated with DM or ASIM over time, the mechanism of action of or response to daxudilimab administration, or the pathogenesis of DM or ASIM.

[0292] skin swab Skin swabs will be collected to profile the skin microbiome at baseline and over time to explore associations with clinical response following daxudilimab treatment.

[0293] Genetics DNA can be isolated from a sample of standard collected whole blood and tested for: Single nucleotide polymorphisms in genes that may be associated with the development of DM or ASIM Fcγ receptors in genes that may be related to the mechanism of action of daxudilimab.

[0294] If DNA extraction fails, a replacement genetic blood sample can be requested from the participant.

[0295] Immunogenicity studies Antibodies to daxudilimab can be investigated by the incidence and titers of ADA in serum samples collected from all participants according to Table 3.

[0296] Additionally, serum samples can be collected at the final visit from participants who discontinue IP or are withdrawn from the study.

[0297] Serum samples can be screened for antibody binding to daxudilimab, and titers of confirmed positive samples can be reported. Other analyses can be performed to verify the stability of antibodies to daxudilimab and / or to further characterize the immunogenicity of daxudilimab.

[0298] Detection and characterization of antibodies to daxudilimab can be performed using validated assays. The ability of the antibodies to neutralize the activity of daxudilimab can be further characterized and / or assessed.

[0299] Samples may be stored for up to 15 years (or according to local regulations) after the last participant's last visit for the study.

[0300] Quality of life The planned time points for all quality of life surveys are shown in Table 3.

[0301] Quality of life can be investigated by: HAQ-DI and PtGDA (part of TIS) SF36 Skindex16 score PROMIS Fatigue 8a PROMIS Physical Function 8b PROMIS Pain Intervention 6a ·PROMIS dyspnea severity ·All pain 24 hour NRS ·PGIC

[0302] HAQ-DI and PtGDA (part of TIS) HAQ DI and PtGDA are described as previously disclosed herein.

[0303] SF36 The SF36 is a health survey that measures functional health and well-being from the participant's perspective with questions across eight health domains: physical functioning, role-physical, bodily pain, overall health, vitality, social functioning, role-emotional, and mental health.

[0304] Skindex16 score The Skindex16 score (Table 11) is used to have participants rate skin conditions that occurred over the previous week (Atherton et al., 2012; Chren et al., 2001). It is a short 16-item survey that uses a numeric analog scale ranging from 0 ("not bothered at all") to 6 ("always bothered"). Responses to the Skindex16 are categorized into three subscales: symptomatic, emotional, and functional.

[0305] [Table 11]

[0306] PROMIS Fatigue 8a PROMIS Fatigue 8a explores a wide range of self-reported symptoms, from a mild subjective feeling of tiredness to an overwhelming, debilitating, and persistent feeling of fatigue that may reduce one's ability to perform daily activities and function normally in household or social roles. Fatigue is divided into the experience of fatigue (frequency, duration, and intensity) and its impact on physical, mental, and social activities. In total, fatigue over the past 7 days is explored.

[0307] PROMIS Physical Function 8b PROMIS Physical Function 8b (V 2.0) measures self-reported ability rather than actual physical activity performance. This includes the participant's upper extremities (dexterity), lower extremities (walking or locomotion), and central areas (neck, back), as well as functionality in useful activities of daily living, such as running errands. The form examines current time periods, rather than functioning over specific time periods.

[0308] PROMIS Pain Interference 6a The PROMIS Pain Interference 6a (V 1.0) assesses the consequences of pain on relevant aspects of participants' life, including the extent to which pain interferes with engaging in social, cognitive, emotional, physical, and restful activities.

[0309] PROMIS dyspnea severity The PROMIS Dyspnea Severity Scale assesses the severity of shortness of breath, or dyspnea, experienced by adults in response to various specific activities (e.g., being able to dress oneself without assistance, preparing a meal, walking 20 steps).

[0310] Total pain 24 hour NRS The 24-hour Total Pain NRS is an 11-point scale scored from 0 (no pain) to 10 (the most intense pain imaginable). Participants select the value that best matches the intensity of the pain they have experienced in the past 24 hours.

[0311] PGIC The PGIC is a categorical scale (Table 12) in which participants are asked to indicate how much their DM or ASIM has changed since starting IP, ranging from "much better" to "no change" to "much worse."

[0312] [Table 12]

[0313] Primary efficacy endpoint analysis The estimand of primary interest is defined as follows, using a hypothetical policy to deal with the intervening event of rescue medication and a treatment policy to deal with the intervening event of treatment discontinuation: 1. Population: Participants in the entire analysis set. 2. Variable (evaluation item): TIS at 24 weeks. 3. Intervening events: Rescue Medications: Data collected after the administration of rescue medication may be excluded. b. Treatment Discontinuation: Participants who discontinue IP before week 24 may be asked to return for scheduled assessments up to week 24. Data collected after discontinuation of IP may be included in the analysis. 4. Population-level summary: mean difference between daxudilimab and placebo.

[0314] Within each of the DM and ASIM populations, the primary efficacy endpoint of TIS at 24 weeks can be analyzed using mixed models for repeated measures (MMRM), with treatment, visit, visit by treatment interaction, and random stratification factors included in the model.

[0315] Supplementary analyses of primary efficacy endpoints Supplemental analyses for the primary efficacy endpoint may be performed using treatment policies to address rescue medication intervening events (i.e., including data collected after rescue medication). Additional analyses may be performed. Details will be described in the SAP.

[0316] Secondary efficacy endpoint analysis For both the DM and ASIM populations, the estimands for the binary secondary efficacy endpoints will be defined as follows, using a composite variability policy to address intervening events: 1. Collective: a.TIS40: Participants in the full analysis set. a.TIS20: Participants in the full analysis set. b. Clinically meaningful reduction in oral corticosteroids (OCS): Participants in the full analysis set with an OCS dose of ≥ 10 mg prednisone or equivalent at baseline 2. Variables (evaluation items): a. TIS40 at 24 weeks is defined as meeting the following criteria: i. TIS score of 40 or more at 24 weeks ii. No deterioration at 2 consecutive visits at or before 24 weeks. iii. No rescue medication use prior to 24 weeks. iv. No discontinuation of IP before 24 weeks. b. TIS20 at 24 weeks is defined as meeting the following criteria: i. TIS score of 20 or more at 24 weeks ii. No deterioration at 2 consecutive visits at or before 24 weeks. iii. No rescue medication use prior to 24 weeks. iv. No discontinuation of IP before 24 weeks. c. A clinically meaningful reduction in OCS at 24 weeks is defined as meeting the following criteria: i. A 25% reduction from baseline at 24 weeks or an OCS dose of 7.5 mg / day prednisone or equivalent. ii. No rescue medication use prior to 24 weeks. iii. No discontinuation of IP before 24 weeks. 3. Intervening events: Rescue medication: captured in variable definition. b. Treatment interruption: captured in variable definition. 4. Population-level summary: Difference in responder rates between daxudilimab and placebo.

[0317] Within each of the DM and ASIM populations, binary secondary endpoints can be analyzed using logistic regression models with treatment, baseline OCS dose (OCS reduction endpoint only), and randomization stratification factors included in the model.

[0318] For both the DM and ASIM populations, using a hypothetical strategy to address the intervening event of rescue medication and a treatment policy to address the intervening event of treatment discontinuation, the estimates for the continuous secondary endpoint of change from baseline to week 24 in CDASI activity score are similar to the primary estimate.

[0319] Within each of the DM and ASIM populations, the change from baseline to week 24 in CDASI activity score can be analyzed using the MMRM approach, with treatment, visit, visit by treatment interaction, random stratification factor included in the model, and baseline CDASI activity score. Missing data can be handled by the MMRM approach.

[0320] Pharmacokinetic analysis PK analyses can be based on the PK analysis set. Descriptive statistics of serum daxudilimab concentrations will be tabulated by visit and by treatment group for each of the DM and ASIM populations. Exposure-response (PD, ADA, safety, and efficacy) analyses can be performed using serum daxudilimab concentrations and available drug response data at the sponsor's discretion and as data permit.

[0321] Pharmacodynamic analysis Results for pDC levels, serum IFN-α, blood MxA, and IFN gene signature levels, and changes from these baselines, can be summarized descriptively by visit and by treatment group using the safety analysis set.

[0322] Immunogenicity analysis The number and percentage of participants developing a positive ADA and developing a positive ADA titer can be summarized by treatment group and by visit for each of the DM and ASIM populations using the safety analysis set.

[0323] Exploratory analysis Exploratory endpoint analyses can be detailed in the SAP.

[0324] Planning Analysis Main analysis The primary analyses can be performed separately for each of the DM and ASIM populations after the last participant completes the 24-week visit or, for that population, is withdrawn from the study before week 24. For the primary analyses, all efficacy and safety data collected before the data cutoff will be analyzed.

[0325] Final analysis Final analyses can be performed separately for each of the DM and ASIM populations after the last participant completes the study or, for that population, is prematurely withdrawn from the study.

[0326] Sample size determination A sample size of approximately 48 participants (maximum of approximately 24 participants per treatment group) is planned for each population (DM and ASIM). Sample size was calculated based on the primary efficacy endpoint. 21 participants per treatment group will provide approximately 80% power to detect a mean difference of 20 between daxuzilimab and placebo using a two-sided t-test at a two-sided alpha level of 0.10, with an estimated standard deviation of 25. The minimum detectable difference between the daxuzilimab and placebo groups is 13. Assuming a 10% dropout rate during the first 24 weeks of the study, approximately 24 participants per treatment group are required.

[0327] Incorporation by Reference All references, articles, publications, patents, patent publications, and patent applications cited herein are incorporated by reference in their entirety for all purposes. However, no reference, article, publication, patent, patent publication, or patent application cited herein is, and should not be, taken as an admission or any form of suggestion that it constitutes valid prior art or forms part of the common general knowledge anywhere in the world.

Claims

1. A method for treating dermatomyositis, the method comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of treatment for dermatomyositis, wherein the ILT7 antibody or antigen-binding fragment thereof comprises complementarity-determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, and the HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, the effective amount is approximately 300 mg, and the antibody is administered once every four weeks.

2. 10. The method of claim 1, wherein the dermatomyositis comprises well-defined myositis.

3. 10. The method of claim 1, wherein the dermatomyositis comprises probable myositis.

4. 1. A method for treating anti-synthetase inflammatory myositis (ASIM), the method comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or an antigen-binding fragment thereof to a subject in need of treatment for ASIM, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, and the HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, the effective amount is approximately 300 mg, and the antibody is administered once every four weeks.

5. 5. The method of claim 4, wherein the ASIM includes well-defined myositis.

6. 5. The method of claim 4, wherein the ASIM includes possible myositis.

7. 1. A method for treating a disease, the method comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or an antigen-binding fragment thereof to a subject in need of disease treatment, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, and the HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, the effective amount comprising a dose from about 300 mg, and the administering is completed once every four weeks.

8. 1. A method for treating a disease, the method comprising administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of disease treatment, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein the HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and wherein the administration is effective to alleviate the disease as compared to (a) a disease in an otherwise comparable subject lacking the administration, or (b) a baseline measure of the disease in the subject in need of disease treatment.

9. 9. The method of any one of claims 1-8, wherein the administering continues for at least about 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, or up to about 15 administrations.

10. 10. The method of any one of claims 8-9, wherein the administering is (b) effective in alleviating disease in the subject in need of treatment, as compared to a baseline measure of the disease, wherein the alleviation is measured by detecting a decreased total improvement score in the subject.

11. 10. The method of any one of claims 8-9, wherein the administering is (b) effective in alleviating disease in the subject in need of treatment, as compared to a baseline measure of the disease, wherein the alleviation is measured by detecting a reduced level of a symptom of the disease in the subject.

12. The method of any one of claims 10 to 11, wherein the measurements are completed after 6 and / or 12 administrations.

13. 13. The method of any one of claims 10 to 12, wherein the total improvement score is 10 or more, 20 or more, 30 or more, 40 or more, or 50 or more.

14. 14. The method of claim 13, wherein the total improvement score is 20 or greater.

15. 14. The method of claim 13, wherein the total improvement score is 40 or greater.

16. 16. The method of any one of claims 11-15, wherein the level of reduction in the symptoms comprises a decrease in a score on a survey selected from the group consisting of: Dermatomyositis Disease Area and Severity Index (CDASI), Myositis Disease Activity Assessment Tool (MDAAT), Myositis Damage Index (MDI), and combinations thereof, compared to baseline.

17. 16. The method of any one of claims 11 to 15, wherein the reduced level of the symptom comprises an increase in lung compliance as measured by an increase in forced vital capacity (FVC) compared to baseline.

18. 10. The method of any one of claims 8-9, wherein the administering is (b) effective in reducing disease in the subject in need of treatment, as compared to a baseline measure of disease, wherein the reduction is measured by a reduction in corticosteroid use, and wherein the measurement is completed after six administrations.

19. 19. The method of claim 18, wherein the corticosteroid comprises prednisone.

20. 20. The method of any one of claims 18-19, wherein the reduction comprises a decrease in frequency of use of at least about 10%, 15%, 20%, 25%, 30%, or 40%.

21. 20. The method of any one of claims 18-19, wherein the reduction is from a dose of about 10 mg / day or more to a dose of 7.5 mg / day.

22. 22. The method of any one of claims 1 to 21, wherein the ILT7 antibody comprises a heavy chain variable region (VH) and a light chain variable region (VL), wherein the VH and VL regions comprise amino acid sequences that are at least 80% identical to SEQ ID NO:2 and SEQ ID NO:7, respectively.

23. 23. The method of any one of claims 1 to 22, wherein the ILT7 antibody comprises a heavy chain variable region (VH) and a light chain variable region (VL), wherein the VH and VL regions comprise the amino acid sequences of SEQ ID NO: 2 and SEQ ID NO: 7, respectively.

24. The method of any one of claims 1 to 23, wherein the ILT7 antibody is a monoclonal antibody.

25. The method of any one of claims 1 to 24, wherein the ILT7 antibody is non-fucosylated.

26. The method of any one of claims 4 to 25, wherein the required subject sample is positive for one or more ASIM-related antibodies.

27. 27. The method of claim 26, wherein the one or more ASIM-associated antibodies are selected from the group consisting of anti-Jo-1, anti-PL-12, anti-PL-7, anti-KS, anti-EJ, anti-OJ, anti-ZO, and anti-YRS(HA).

28. 28. The method of any one of claims 1 to 27, wherein the subject in need thereof has a dermatomyositis-associated rash.

29. The method of any one of claims 1 to 28, wherein the disease is caused by plasmacytoid dendritic cells.

30. 30. The method of any one of claims 1-29, wherein said administering is effective in reducing the level of type I interferon in said subject compared to the subject's baseline level.

31. 31. The method of claim 30, wherein the level is reduced by at least about 1-fold, 5-fold, 10-fold, 25-fold, 50-fold, 75-fold, 100-fold, 150-fold, or 250-fold.

32. 32. The method of any one of claims 7-31, wherein said administering is effective in eliminating said disease for at least about 3 months, 6 months, 1 year, or 2 years.

33. The method of any one of claims 1 to 32, wherein the administration is subcutaneous.

34. A method for treating dermatomyositis, the method comprising subcutaneously administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or antigen-binding fragment thereof to a subject in need of treatment for dermatomyositis, wherein the ILT7 antibody or antigen-binding fragment thereof comprises complementarity-determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, wherein the HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and the effective amount is approximately 300 mg.

35. A method for treating anti-synthetase inflammatory myositis (ASIM), the method comprising subcutaneously administering an effective amount of an anti-immunoglobulin-like transcript 7 (ILT7) antibody or an antigen-binding fragment thereof to a subject in need of treatment for ASIM, wherein the ILT7 antibody comprises complementarity determining regions (CDRs) HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3, and the HCDR1, HCDR2, HCDR3, LCDR1, LCDR2, and LCDR3 comprise the amino acid sequences of SEQ ID NOs: 3, 4, 5, 8, 9, and 10, respectively, and the effective amount is approximately 300 mg.

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