Advances in therapy for ANCA-associated vasculitis.

The anti-neutrophil cytoplasmic antibody-associated vasculitides include granulomatosis with polyangiitis (Wegener's granulomatosis) and microscopic polyangiitis. The introduction of therapy with cytotoxic agents such as cyclophosphamide transformed these diseases from fatal diagnoses to chronic conditions characterized by cycles of relapse and remission. Modern treatment strategies have focused on minimizing cyclophosphamide exposure or eliminating its use altogether. Two randomized clinical trials have shown that rituximab is not inferior to cyclophosphamide for induction of remission in patients with severe granulomatosis with polyangiitis (Wegener's) or microscopic polyangiitis. For patients with non-life threatening disease, methotrexate may be used to induce and maintain remission, although some patients may have a higher long-term risk of relapse as a result. For patients with life-threatening disease, plasma exchange may be an effective adjuvant therapy. This article reviews seminal studies from the past decade that have contributed to the current standard of care.