Therapeutic Complement Targeting in ANCA-Associated Vasculitides and Thrombotic Microangiopathy.

ABSTRACT: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of systemic autoimmune disorders characterized by necrotizing inflammation of medium-to-small vessels, a relative paucity of immune deposits, and an association with detectable circulating ANCAs. AAVs include granulomatosis with polyangiitis (renamed from Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Until recently, AAVs have not been viewed as complement-mediated disorders. However, recent findings predominantly from animal studies demonstrated a crucial role of the complement system in the pathogenesis of AAVs. Complement activation or defects in its regulation have been described in an increasing number of acquired or g

SUBMITTER: Novikov P 

PROVIDER: S-EPMC6945915 | biostudies-literature | 2016 Sep-Dec

REPOSITORIES: biostudies-literature