Rituximab with or without a conventional maintenance agent in the treatment of relapsing granulomatosis with polyangiitis (Wegener's): a retrospective single-center study.

To evaluate the efficacy and safety of rituximab (RTX) induction therapy and the duration of remission, when RTX is used with or without a conventional maintenance agent, in a cohort of patients with granulomatosis with polyangiitis (Wegener's) (GPA). This was a retrospective, single-center study of patients with relapsing GPA treated with at least 1 course of RTX (4 weekly doses of 375 mg/m(2) intravenously [IV] or 2 fixed doses of 1,000 mg IV 2 weeks apart). Complete remission was defined as the absence of disease activity measured by a Birmingham Vasculitis Activity Score for Wegener's granulomatosis of 0 and not qualified by the prednisone dosage at the time. Eighty-nine patients achieved remission after their first course of RTX and were not re-treated preemptively with RTX to maintain remission of their disease during followup. Among these patients, relapse-free survival was significantly higher in those who received a conventional maintenance agent (azathioprine, methotrexate, or mycophenolate mofetil) in conjunction with RTX and glucocorticoids (n = 47) than in those who received no additional immunosuppressive agent (n = 42) (P = 0.04). The hazard ratio of relapse in those receiving a maintenance agent was 0.53 (95% confidence interval 0.29-0.97). Serious adverse events did not differ between the 2 groups. Within a subset of 15 patients in the cohort who were relapse free 2 years after 1 course of RTX, remissions endured for 2-6 years in 8 patients. RTX is an effective remission-inducing agent in GPA. The addition of a conventional maintenance agent to RTX and glucocorticoids decreased the incidence of relapse and did not result in a higher incidence of adverse events.