Current landscape of antineutrophil cytoplasmic antibody-associated vasculitis: classification, diagnosis, and treatment

Abstract

This article provides an update on the diagnosis and management of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, granulomatosis with polyangiitis (formerly Wegener), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss). Focus is on new schemes of classification and the importance of ANCAs in the diagnosis and prognosis of these systemic vasculitides. Current therapeutic strategies consisting of glucocorticoids in conjunction with conventional or biologic agents for both induction of remission and remission maintenance are outlined. Future research directions include investigation of the optimal duration and frequency of maintenance therapy and development of targeted therapeutic agents.

Keywords: ANCA-associated vasculitis; Eosinophilic granulomatosis with polyangiitis (Churg-Strauss); Granulomatosis with polyangiitis (Wegener); Microscopic polyangiitis.