Evidence-based therapy for the ANCA-associated vasculitides: what do the trials show so far?

Anti-neutrophil cytoplasm antibody-associated vasculitides are rare multisystem inflammatory diseases including granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Previously these diseases were almost universally fatal, however, with current therapy 5-year survival for microscopic polyangiitis and granulomatosis with polyangiitis is approximately 80%. This review discusses the recent randomized controlled trials and other studies that have driven the improvement in survival and also highlights treatment-related morbidity and areas of unmet need in the management of anti-neutrophil cytoplasm antibody-associated vasculitides.