Treatment of ANCA-Associated Vasculitides

Abstract

A subgroup of systemic necrotizing vasculitides, antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAVs), are known to have circulating ANCA in >30–90% of the affected patients. Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg–Strauss syndrome), and microscopic polyangiitis (MPA) are AAVs [1]. Renal-limited vasculitis, another AAV, is a bit of a question mark, as it is a disease that initially affects the kidney but could then evolve to become MPA or GPA. All AAVs affect small-sized vessels, and ANCA are thought to play a role in their pathogeneses. Due to our improved understanding of AAV pathophysiological mechanisms and identification of their cellular and molecular targets, AAV treatments are now more specific than they had been in the past.