REVIEW Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab

Abstract Granulomatosis with polyangiitis and micro-scopic polyangiitis are anti-neutrophil cytoplasmic anti-body-associated vasculitides (AAVs) that are prone to cycles of remission and relapse. The introduction of cyto-toxic therapy has changed the prognosis for these diseases from typically fatal to manageable chronic illnesses with a relapsing course. Despite improvements in outcomes, recurrence of disease and drug-related toxicity continue to produce significant morbidity and mortality. Better under-standing of the pathogenesis of AAV and the mechanism of action of cyclophosphamide has led to investigation of therapies that target B cells. Two randomized controlled trials have shown that rituximab is not inferior to cyclo-phosphamide for induction of remission in severe AAV, with no significant difference in the incidence of overall adverse events in rituximab- versus cyclophosphamide-treated patients. Data from ongoing clinical trials will determine the role of rituximab in the maintenance of remission