Summary

A few decades ago, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitide—granulomatosis with polyangiitis, polyangiitis, and microscopic polyangiitis—were associated with high rates of mortality. Tremendous strides have since been made in treating these diseases. The use of less toxic immunosuppressant drugs has improved the outcomes of patients, and, in recent years, the development of biologic agents and a better understanding of disease pathogenesis have contributed to the discovery of rituximab as an effective alternative to the immunosuppressive cyclophosphamide to induce remission in ANCA-associated vasculitis.