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Therapeutic complement targeting in ANCA-associated vasculitides and thrombotic microangiopathy

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Item Type:Review
Title:Therapeutic complement targeting in ANCA-associated vasculitides and thrombotic microangiopathy
Creators Name:Novikov, P. and Kozlovskaya, N. and Moiseev, S. and Shilov, E. and Bobkova, I. and Schreiber, A. and Tsvetkov, D. and Gollasch, M. and Mah, N. and El Amrani, K. and Kurtz, A.
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 genetically driven forms of thrombotic microangiopathy. Coinciding with this expanding spectrum of complement-mediated diseases, the question arises as to which AAV patients might benefit from a complement-targeted therapy. Therapies directed against the complement system point to the necessity of a genetic workup of genes of complement components and regulators in patients with AAV. Genetic testing together with pluripotent stem cells and bioinformatics tools may broaden our approach to the treatment of patients with aggressive forms of AAV.
Keywords:ANCA-Associated Vasculitis, C5b-9, Opsonins, CCX168, C5a Receptor, Human Pluripotent Stem Cells, Genetic Testing, Complement
Source:Biomedicine Hub
Page Range:453106
Date:13 December 2016
Official Publication:https://doi.org/10.1159/000453106
PubMed:View item in PubMed

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