45 results about "Vasculitis" patented technology

The invention provides an application of a reagent for detecting an effector CD4 + T cell in peripheral blood of a subject in preparation of a product for diagnosing membranous nephropathy, lupus nephritis or ANCA-related vasculitis. The invention also provides an application of the effector CD4 + T cell in peripheral blood in preparation of a diagnostic model for diagnosing membranous nephropathy, lupus nephritis or ANCA-related vasculitis. The marker is detected through flow cytometry, and the result shows that the Ejector CD4 + T cell is remarkably increased in a patient with membranous nephropathy, can effectively distinguish membranous nephropathy from healthy control (verification set AUC 0.8098), can assist in identifying membranous nephropathy, lupus nephritis (verification set AUC 0.8686) and ANCA-related vasculitis (verification set AUC 0.8968), and the level of the Ejector CD4 + T cell is related to whether membranous nephropathy is difficult to treat or not.

This invention relates to the fields of biomedical technology and biological model construction technology, and discloses an ANCA-associated vasculitis glomerular endothelial cell injury model, its construction method and application, and the application of BACH1 inhibitors. The method includes: (1) activating neutrophils sequentially with tumor necrosis factor α and immunoglobulin G to obtain a culture medium containing activated neutrophils; wherein the immunoglobulin G is immunoglobulin G isolated and purified from the plasma of patients with ANCA-associated vasculitis; (2) co-culturing the culture medium containing activated neutrophils with human glomerular endothelial cells to obtain the cell injury model. The cell injury model provided by this invention can verify the role of neutrophil adhesion characteristics in endothelial cell injury, and at the same time, the cell injury model can better simulate the immune microenvironment of ANCA-associated vasculitis glomerular endothelial cells in vivo.

The application belongs to the technical field of biological detection, and discloses a cardiovascular marker control article and a preparation method thereof. The cardiovascular marker control article comprises a serum matrix, antigens, a preservative and a stabilizer. The antigens include cardiac troponin I antigens, creatine kinase isoenzyme antigens, myoglobin antigens, amino-terminal brain natriuretic peptide precursor antigens, brain natriuretic peptide antigens, D-dimer antigens, heart-type fatty acid-binding protein antigens, myeloperoxidase antigens and lipoprotein phospholipase A2 antigens. The stabilizer includes at least one of D-trehalose, sucrose, glycine, inositol and polyethylene glycol. The cardiovascular marker control article can cover various cardiovascular disease markers, including heart failure, pulmonary embolism, vasculitis and atherosclerosis, myocardial injury, myocardial infarction and the like. The control article can be used as a third-party control article for indoor and inter-laboratory quality control of a cardiovascular disease screening and auxiliary diagnosis measurement system.

The invention discloses a diagnostic marker for ANCA (Angiovasculitis) related vasculitis. It is found for the first time that the CD19 + CD38 + CD27-CD24 + cell can be used as the diagnosis marker of ANCA related vasculitis, and the diagnosis efficiency is high; in addition, it is found that the CD19 + CD38 + CD27-CD24 + cells can remarkably distinguish ANCA related vasculitis and membranous nephropathy or ANCA related vasculitis and lupus nephritis, a new direction is provided for diagnosis and subsequent treatment of ANCA related vasculitis, and the application has wide application prospects clinically.

Provided herein are methods of treating ANCA-associated vasculitis (AAV) in individuals in need thereof comprising administering a complement component 5a receptor (C5aR) antagonist. Also provided herein are methods of treating a ANCA-associated vasculitis (AAV) with renal involvement in an individual in need thereof comprising administering a complement component 5a receptor (C5aR) antagonist to the individual if the individual exhibits an elevated urinary soluble CD163 (sCD163) to creatinine ratio compared to individuals without AAV. In some embodiments, the complement component 5a receptor (C5aR) antagonist is avacopan.

This disclosure relates to RNAi agents capable of inhibiting complement factor B (CFB) gene expression. Pharmaceutical compositions containing CFB RNAi agents and methods of use thereof are also disclosed. The CFB RNAi agents disclosed herein may be conjugated to a targeted ligand containing an N-acetyl-galactosamine ligand to facilitate in vivo delivery to hepatocytes. RNAi agents can be used in methods of treating diseases, disorders, or conditions partially mediated by CFB gene expression, including IgA nephropathy (IgAN), C3 glomerulopathy (C3G), immune complex-mediated membrane proliferative glomerulonephritis (IC-MPGN), lupus nephritis (LN), anti-glomerular basement membrane antibody disease (anti-GBM), ischemia-reperfusion injury and T-cell-mediated rejection in kidney transplantation (TCMR), anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, age-related macular degeneration (AMD) including early and / or intermediate-stage AMD, geographic atrophy (GA), glaucoma, Doyne honeycomb retinal dystrophy, paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), pre-eclampsia, rheumatoid arthritis (RA), and / or other complement-mediated disorders.

The present invention relates to a new therapeutic approach for treatment of ANCA-associated vasculitis (Anti-neutrophil cytoplasmic autoantibody-associated vasculitis, AAV). It provides an agent for reducing expression of proteinase 3 (PR3) in a cell, in particular, a gene editing compound targeting the proteinase 3 gene, an epigenetic editing compound targeting the proteinase 3 gene, or an expression vector encoding an inhibitory oligonucleotide capable of selectively binding to a target nucleic acid sequence in mRNA encoding proteinase 3, e.g., an inhibitory oligonucleotide capable of inducing RNAi or an antisense oligonucleotide. Further, a method of reducing expression of proteinase 3 in a hematopoietic stem cell or cell derived therefrom, e.g., a neutrophil, is provided, as well as such cells having a reduced expression of proteinase 3. The cells or agents can be used for treating a subject having a proteinase 3-ANCA-associated vasculitis.

The present invention provides an antibody or a conjugated fragment of a citrulline-containing epitope used for the treatment or prevention of diseases associated with the release of extracellular traps from cells, such as neutrophil extracellular trap (NET)-associated pathology (NET-associated pathology) or eosinophil extracellular trap (EET)-associated pathology (EET-associated pathology), and provides a method comprising administering at least one dose of the antibody at a specific concentration. The present invention also provides the method itself. NET-related pathologies include systemic lupus erythematosus (SLE), lupus, sepsis, vasculitis, inflammatory arthritis, rheumatoid arthritis and osteoarthritis, psoriasis, Alzheimer's disease, autoimmune hepatitis, juvenile idiopathic arthritis, myositis (polymyositis and dermatomyositis), Sjögren's disease, antiphospholipid syndrome, Behçet's disease, spondylitis, spondyloarthritis, multiple system atrophy, Parkinson's disease, Lewy body dementia, asthma, allergic rhinovirus exacerbated asthma, allergic asthma, acute respiratory distress syndrome, cystic fibrosis, fibrosis and idiopathic pulmonary fibrosis, heart failure, atherosclerosis, dry eye disease, uveitis, non-granulomatous uveitis, granulomatous uveitis, dermatitis, atopic dermatitis, COPD, bronchitis, or wound healing in diabetes, cancer, cancer metastasis, or wound healing in diabetes, cancer, cancer metastasis, and in This includes other NET-related pathologies, such as the health of transplanted organs in vivo or ex vivo.The present invention also relates to wound healing in SLE, lupus, sepsis, vasculitis, inflammatory arthritis, rheumatoid arthritis and osteoarthritis, psoriasis, Alzheimer's disease, autoimmune hepatitis, juvenile idiopathic arthritis, myositis (polymyositis and dermatomyositis), Sjögren's disease, antiphospholipid antibody syndrome, Behçet's disease, spondylitis, spondyloarthritis, multiple system atrophy, Parkinson's disease, Lewy body dementia asthma, allergic rhinovirus exacerbating asthma, allergic asthma, acute respiratory distress syndrome, cystic fibrosis, fibrosis and idiopathic pulmonary fibrosis, heart failure, atherosclerosis, dry eye disease, uveitis, non-granulomatous uveitis, granulomatous uveitis, dermatitis, atopic dermatitis, COPD, bronchitis, thrombotic disease, cardiovascular disease, or diabetes, cancer, cancer metastasis, or wound healing in diabetes, cancer, cancer metastasis, and in vivo or ex vivo The present invention provides pharmaceutical compositions and methods for treating and preventing NET-related pathologies, including other NET-related pathologies such as the health of transplanted organs in vivo. NET-related pathologies include eosinophilic diseases or conditions of the skin, respiratory eosinophilic diseases or conditions, gastrointestinal eosinophilic diseases or conditions, allergic diseases or conditions, or eosinophilic diseases or conditions such as helminthic, fungal, viral or bacterial infections.

The present invention relates to the dipeptidyl-peptidase I (DPPI or Cathepsin C) inhibitor of formula (I), which is useful in the prevention and / or treatment of bronchiectasis with certain underlying aetiologies, in the prevention and / or treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis or hidradenitis suppurativa using therapeutically efficacious dosages and to polymorphs of Compound (I) as well as to pharmaceutical compositions comprising Compound (I) and its polymorphs.

The invention provides an antibody specifically binding to RA33 or an antigen binding fragment thereof as well as a preparation method and application thereof, and relates to the technical field of biology. The antibody capable of specifically binding to RA33 or the antigen binding fragment of the antibody comprises a heavy chain variable region and a light chain variable region, the heavy chain variable region comprises a complementary determining region VH-CDR1, a complementary determining region VH-CDR2 and a complementary determining region VH-CDR3; the light chain variable region comprises a complementary determining region VL-CDR1, a complementary determining region VL-CDR2 and a complementary determining region VL-CDR3; the amino acid sequence of the complementarity determining region is specifically defined. The antibody or the antigen binding fragment thereof can effectively bind to the RA33 antigen, and can be used for detection of the RA33 antigen and detection of vasculitis diseases.

The present invention provides an antibody comprising citrulline epitope or a binding fragment thereof for use in the treatment or prevention of a disease associated with release of an extracellular trap from a cell, such as a neutrophil extracellular trap (NET)-associated disease (NET-associated disease) or an eosinophil extracellular trap (EET)-associated disease (EET-associated disease), the method comprises administering at least one dose of an antibody at a specific concentration. The present invention also provides these methods themselves. NET related diseases include systemic lupus erythematosus (SLE), lupus, septicemia, vasculitis, inflammatory arthritis, rheumatoid arthritis and osteoarthritis, psoriasis, Alzheimer's disease, autoimmune hepatitis, juvenile idiopathic arthritis, myositis (polymyositis and dermatomyositis), sicca syndrome, antiphospholipid syndrome, Behcet's disease, spondylitis, spondyloarthropathy, multi-system atrophy, and the like. Parkinson's disease, Lewy body dementia, asthma, allergic rhinovirus aggravated asthma, allergic asthma, acute respiratory distress syndrome, cystic fibrosis, fibrosis and idiopathic pulmonary fibrosis, heart failure, atherosclerosis, dry eye disease, uveitis, non-granulogenic uveitis, granulogenic uveitis, dermatitis, atopic dermatitis, COPD, bronchitis, and the like. Or other NET-related diseases, such as wound healing in diabetic patients, cancer, cancer metastasis, and transplanted organ health in vivo or in vitro. The invention also provides a pharmaceutical composition and a method for treating or preventing NET related diseases. Such as SLE, lupus, sepsis, vasculitis, inflammatory arthritis, rheumatoid arthritis and osteoarthritis, psoriasis, Alzheimer's disease, autoimmune hepatitis, juvenile idiopathic arthritis, myositis (polymyositis and dermatomyositis), sicca syndrome, antiphospholipid syndrome, Behcet's disease, spondylitis, spondyloarthropathy, multi-system atrophy, Parkinson's disease, Lewy body dementia, asthma, and the like. Allergic rhinovirus aggravated asthma, allergic asthma, acute respiratory distress syndrome, cystic fibrosis, fibrosis and idiopathic pulmonary fibrosis, heart failure, atherosclerosis, dry eye disease, uveitis, non-granulometric uveitis, granulometric uveitis, dermatitis, atopic dermatitis, COPD, bronchitis, thrombotic disease, cardiovascular disease, and the like. Or other NET-related diseases, such as wound healing in diabetic patients, cancer, cancer metastasis, and transplanted organ health in vivo or in vitro. The EET-related diseases include eosinophilic granulocyte diseases or conditions, such as eosinophilic granulocyte diseases or conditions of skin; a respiratory eosinophilic granulocyte disease or condition; gastrointestinal eosinophilic cell diseases or disorders; allergic diseases or conditions; or worm, fungus, virus or bacterial infection.

The present disclosure relates to RNAi agents able to inhibit Complement Factor B (CFB) gene expression. Also disclosed are pharmaceutical compositions that include CFB RNAi agents and methods of use thereof. The CFB RNAi agents disclosed herein may be conjugated to targeting ligands, including ligands that comprise N-acetyl-galactosamine, to facilitate the in vivo delivery to hepatocyte cells. The RNAi agents can be used in methods of treatment of diseases, disorders, or symptoms mediated in part by CFB gene expression, including IgA nephropathy (IgAN), C3 glomerulopathy (C3G), immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN), lupus nephritis (LN), Anti-Glomerular Basement Membrane disease (anti-GBM), ischemia reperfusion injury and T-cell mediated rejection (TCMR) in kidney transplantation, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, age-related macular degeneration (AMD), including early and / or intermediate AMD, geographic atrophy (GA), glaucoma, Doyne honeycomb retinal dystrophy, paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), pre-eclampsia, rheumatoid arthritis (RA), and / or other complement-mediated diseases.

The invention belongs to the technical field of biological model construction, and particularly relates to a method for constructing a vasculitis model in zebra fish. The compound is obtained by inducing a Candida albicans water soluble substance (CAWS), and the specific method is micro-injection or soaking. According to the method, whether modeling succeeds or not can be judged in the living body state of the model animal, the method is more suitable for visually observing the inflammation occurrence position, a research method for vasculitis (including Kawasaki disease) is developed, follow-up scientific research data can be richer and more accurate, and a more solid theoretical basis is laid for clinical transformation of targeted therapy of vasculitis (including Kawasaki disease).

The utility model relates to medical instrument technical field relates to a bend intravenous infusion needle, include: infusion needle, infusion pipe and connecting joint connected in proper order, the side of infusion pipe is close to infusion needle is provided with fixed establishment, the side of infusion pipe is close to connecting joint is provided with water stop clamp, the infusion needle includes with infusion pipe connection's curved needle and sets up in the horizontal needle of curved needle tail end, the horizontal needle tail end is provided with the oblique puncture part, the connecting joint includes with infusion pipe connection's connecting seat and sets up in the cap of connecting seat tail end, is provided with the furrow on the cap. The utility model infusion needle adapts human body skin camber, makes needle head and puncture part close more closely, reduces the deviation rate of puncture, arc passivation puncture part will " cut type " puncture is converted into " expansion type " separation, reduces blood vessel wall damage area, blood vessel spasm and thrombosis risk reduces obviously, reduces the incidence rate of blood vessel inflammation after infusion.

This invention discloses a method for improving a mouse model of ANCA-associated vasculitis induced by anti-GBM antibodies, relating to the field of animal model construction. The method involves subcutaneously injecting mice with 20 μg rMPO on Day 0; administering 10 μg rMPO on Day 7; subcutaneously injecting 250 μg / kg GCSF daily from Day 11 to 15; and injecting 100 μl of goat anti-GBM serum via the tail vein on Day 16 to obtain the improved mouse model. This method improves the existing anti-GBM antibody-induced ANCA-associated vasculitis mouse model by injecting GCSF. This model enhances the disease severity in ANCA-associated vasculitis mice, specifically reflecting the disease in renal pathology and proteinuria, making the differences more obvious and effectively improving the success rate of model establishment.

A drug, dosage regimen, administration interval, and mode of administration for the treatment of ANCA-associated vasculitis (AAV) using an effective amount of a drug targeting Blys and / or APRIL. The result shows that the provided effective dose of the drug targeting Blys and / or APRIL shows good safety and treatment effect in the process of treating or relieving ANCA-related vasculitis patients.

This invention provides the use of a reagent for detecting Effector CD4+ T cells in peripheral blood of subjects in the preparation of products for diagnosing membranous nephropathy, lupus nephritis, or ANCA-associated vasculitis. This invention also provides the use of peripheral blood Effector CD4+ T cells in the preparation of diagnostic models for membranous nephropathy, lupus nephritis, or ANCA-associated vasculitis. This invention detects this biomarker by flow cytometry, and the results show that Effector CD4+ T cells are significantly elevated in patients with membranous nephropathy, effectively distinguishing membranous nephropathy from healthy controls (validation set AUC 0.8098), and can help differentiate membranous nephropathy from lupus nephritis (validation set AUC 0.8686) and ANCA-associated vasculitis (validation set AUC 0.8968), and its level is correlated with the refractory nature of membranous nephropathy.

Disclosed in the present invention are a polymorph of a benzo[c]chroman compound, a preparation method therefor and use thereof. The present invention particularly provides a polymorph of a compound represented by formula I, a preparation method therefor and use thereof. The compound represented by formula I is used as a cathepsin C inhibitor, and a crystalline form of the compound and a pharmaceutical composition comprising the crystalline form can be used for treating asthma, obstructive pulmonary diseases, bronchiectasis, ANCA-related vasculitis, psoriasis, α1-antitrypsin deficiency, lupus nephritis, diabetes, inflammatory bowel diseases, rheumatic arthritis, rhinosinusitis, hidradenitis suppurativa or cancers.

A method for testing a diseased state of vasculitis and a method for testing an exacerbation risk of COVID-19, the method including: detecting or quantifying, in a blood sample collected from a subject, (1) a No. 1 APOA2-like protein that causes an antigen-antibody reaction with a single chain variable region fragment consisting of an amino acid sequence set forth in SEQ ID NO: 1 and has a molecular weight of 23 to 25 kDa, and / or (2) a No. 2 APOA2-like protein that causes an antigen-antibody reaction with a single chain variable region fragment consisting of an amino acid sequence set forth in SEQ ID NO: 1 and has a molecular weight of 16 to 20 kDa.

[Problem] Provided are a new marker of disease activity useful for testing a diseased state of vasculitis or an exacerbation risk of COVID-19, and a means for testing a diseased state of vasculitis or an exacerbation risk of COVID-19 using the marker. [Solution] According to an aspect of the present invention, there are provided a method for testing a diseased state of vasculitis and a method for testing an exacerbation risk of COVID-19, the method including: detecting or quantifying, in a blood sample collected from a subject, (1) a No. 1 APOA2-like protein that causes an antigen-antibody reaction with a single chain variable region fragment consisting of an amino acid sequence set forth in SEQ ID NO: 1 and has a molecular weight of 23 to 25 kDa, and / or (2) a No. 2 APOA2-like protein that causes an antigen-antibody reaction with a single chain variable region fragment consisting of an amino acid sequence set forth in SEQ ID NO: 1 and has a molecular weight of 16 to 20 kDa.

The present invention relates to the dipeptidyl-peptidase I (DPPI or Cathepsin C) inhibitor of formula (I), which is useful in the prevention and / or treatment of bronchiectasis with certain underlying aetiologies, in the prevention and / or treatment of anti-neutrophil cytoplasmic anti- body-associated vasculitis or hidradenitis suppurativa using therapeutically efficacious dosages and to polymorphs of Compound (I) as well as to pharmaceutical compositions comprising Compound (I) and its polymorphs.

The present disclosure relates to RNAi agents able to inhibit Complement Factor B (CFB) gene expression. Also disclosed are pharmaceutical compositions that include CFB RNAi agents and methods of use thereof. The CFB RNAi agents disclosed herein may be conjugated to targeting ligands, including ligands that comprise N-acetyl-galactosamine, to facilitate the in vivo delivery to hepatocyte cells. The RNAi agents can be used in methods of treatment of diseases, disorders, or symptoms mediated in part by CFB gene expression, including IgA nephropathy (IgAN), C3 glomerulopathy (C3G), immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN), lupus nephritis (LN), Anti-Glomerular Basement Membrane disease (anti-GBM), ischemia reperfusion injury and T-cell mediated rejection (TCMR) in kidney transplantation, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, age-related macular degeneration (AMD), including early and / or intermediate AMD, geographic atrophy (GA), glaucoma, Doyne honeycomb retinal dystrophy, paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), pre-eclampsia, rheumatoid arthritis (RA), and / or other complement-mediated diseases.

Disclosed are polymorphs of benzo[c]chromane compounds, and preparation methods and uses thereof, and specifically provided are polymorphs of a compound shown in formula I, and preparation methods and uses thereof. The compound shown in formula I is a cathepsin C inhibitor, and the crystal form and the pharmaceutical composition containing the crystal form can be used for treating asthma, obstructive pulmonary disease, bronchiectasis, ANCA-associated vasculitis, psoriasis, alpha 1-antitrypsin deficiency, lupus nephritis, diabetes, inflammatory bowel disease, rheumatoid arthritis, sinusitis, hidradenitis suppurativa or cancer.

The invention belongs to the technical field of biological medicine, and particularly relates to application of a PLCG2 inhibitor in preparation of a product for treating Kawasaki disease. The effect of the PLCG2 gene in treatment of the Kawasaki disease is verified, and in a Kawasaki disease cell model, after a PLCG2 inhibitor is added, functional damage caused by over-expression of the PLCG2 can be reversed, endothelial cell dysfunction can be relieved, and the vascular inflammatory damage process can be inhibited. Therefore, the PLCG2 inhibitor can be used as a medicine for treating the Kawasaki disease and has potential development prospects.

Provided herein are synthetic polypeptides derived from the high mobility group box 1 (HMGB1) host protein that can disrupt bacterial biofilms and prevent the formation of neutrophil extracellular traps (NETs). Also provided herein are methods for disrupting abnormal or excessive NET formation, particularly suitable for treating high-risk populations, such as those infected with SARS-CoV-2, sepsis, autoimmune diseases (such as systemic lupus erythematosus, rheumatoid arthritis, type 1 diabetes, and small vessel vasculitis), autoinflammatory diseases (such as gout and inflammatory bowel disease), and metabolic diseases (such as type 2 diabetes and obesity).

The present invention relates to the dipeptidyl-peptidase I (DPPI or Cathepsin C) inhibitor of formula (I), which is useful in the prevention and / or treatment of bronchiectasis with certain underlying aetiologies, in the prevention and / or treatment of anti-neutrophil cytoplasmic anti- body-associated vasculitis or hidradenitis suppurativa using therapeutically efficacious dosages and to polymorphs of Compound (I) as well as to pharmaceutical compositions comprising Compound (I) and its polymorphs.

The invention discloses a Tibetan medicine anti-inflammation inunction preparation and a preparation method thereof, and belongs to the technical field of Tibetan medicines, and the Tibetan medicine anti-inflammation inunction preparation comprises rhubarb, alkali, oxytropis verticillata, herba achyranthis bidentatae, stellera chamaejasme, euphorbia macrocarpa, goethite, folium ilicis scandens, rock brain, volundaisy, gypsum rubrum, rhubarb, thalictrum aquilegifolium, codonopsis alpina, Perot, semen cassiae, abelmoschus esculentus seeds and lagotis diffusa. The medicament has the effects of promoting blood circulation to remove blood stasis, dispelling wind, eliminating dampness, relaxing tendons, dredging collaterals, diminishing inflammation and relieving pain. The traditional Chinese medicine composition can be used for treating joint swelling and pain, limb stiffness, limited activity, hand and foot spasm, neuralgia and other symptoms caused by vasculitis, varicosity, peripheral neuritis, rheumatism, rheumatoid arthritis, gout, fracture and the like, and has effective swelling and pain relieving effects.

Provided herein are compositions and methods for treating neuroinflammatory diseases and conditions, the neuroinflammatory diseases and conditions include Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), multiple sclerosis (MS), traumatic brain injury (TBI), complex regional pain syndrome (CRPS), optic neuritis (ON), optic neuromyelitis (NMO), chronic traumatic encephalopathy (CTE), ischemic stroke (IS), vascular dementia (VAD), anti-oligodendroglia glycoprotein antibody disease (MOGAD), and the like. Transverse myelitis (TM), autoimmune encephalitis (AE), acute disseminated encephalomyelitis (ADEM), chronic meningitis (CM), central nervous system (CNS) vasculitis, hashimoto encephalitis (HE), neurosarcoidosis (NS), multi-system atrophy (MSA), migraine, chemotherapy-induced pain or dementia-related pain.

A pharmaceutically acceptable salt of a benzo[c]chroman compound and a polymorphic form and use of the pharmaceutically acceptable salt. The pharmaceutically acceptable salt is selected from hydrochloride, methanesulfonate, phosphate, L-tartrate, maleate, p-toluenesulfonate, sulfate, fumarate, succinate, citrate, malate, and hydrobromide. The pharmaceutically acceptable salt and the polymorphic form thereof improve the bioavailability and the stability, and as a cathepsin C inhibitor, can be used for treating asthma, obstructive pulmonary disease, bronchiectasis, ANCA-associated vasculitis, psoriasis, α1-antitrypsin deficiency, lupus nephritis, diabetes, inflammatory bowel disease, rheumatoid arthritis, sinusitis, hidradenitis suppurativa, or cancer.

This invention relates to a drug for treating ANCA-associated vasculitis (AAV) using an effective dose of the drug, administration regimen, administration interval, and dosage form targeting BlyS and / or APRIL. The provided effective dose of the drug targeting BlyS and / or APRIL has been demonstrated to exhibit good safety and therapeutic efficacy in the treatment or relief of patients with ANCA-associated vasculitis.