The present invention relates to compounds of formula (I) as inhibitors of
mannan-binding
lectin-associated
serine protease-2 (MASP-2), including inhibitors that selectively inhibit MASP-2 over
thrombin, for use in the treatment of e.g. a thrombotic
microangiopathy (TMA), a renal condition, an inflammatory reaction resulting from tissue or
organ transplantation, an
ischemia reperfusion injury, a complication associated with diabetes, a cardiovascular
disease or disorder, an inflammatory
gastrointestinal disorder, a pulmonary disorder, an
ophthalmic disease or disorder, disseminated intravascular coagulation, graft-versus-
host disease, veno-
occlusive disease, diffuse alveolar hemorrhage, idiopathic pneumonia syndrome, capillary leak syndrome, engraftment syndrome, fluid overload, a
traumatic brain injury (TBI) or a combination thereof