Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Is There Still a Role for Cyclophosphamide?

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of systemic necrotizing vasculitides that includes granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. Treatment of these conditions has improved during the past 2 decades with better understanding of these conditions and availability of newer agents. Cyclophosphamide (CYC) was the first drug demonstrated to afford successful treatment and improvement in AAV. With the emergence of newer agents with more favorable safety profiles, CYC is no longer the cornerstone of management of AAV. This article reviews existing data for treatment and the current role of CYC in the management of AAV.