Rituximab With or Without a Conventional Maintenance Agent in the Treatment of Relapsing Granulomatosis With Polyangiitis (Wegener's): A Retrospective Single‐Center Study

Abstract

Objective. 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).Methods. 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.Results. 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, relapsefree 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.Conclusion. RTX is an effective remissioninducing 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.

Updates in ANCA-associated vasculitis

“…gov Identifier: NCT 01697267) is evaluating rituximab, 1000 mg every 4 months (vs azathioprine), for maintenance in relapsing AN-CA-positive patients with GPA or MPA following a glucocorticoid-rituximab-based induction. Because none of these rituximab-based maintenance options have been validated and/or officially approved yet, the remaining choices following rituximab-based induction are, in practice, to retreat (with rituximab) only for clinical relapse or to give a conventional maintenance immunosuppressive agent such as azathioprine or methotrexate (92,98).…”

Refractory optic perineuritis due to granulomatosis with polyangiitis successfully treated with methotrexate and mycophenolate mofetil combination therapy

“…However, relapses are frequent in patients treated with only rituximab (8). It was reported that routine re-treatment with rituximab alone, or in combination with azathioprine or MTX, was effective in preventing relapses (8,9). In our case, the induction therapy with rituximab and azathioprine with PSL 60 mg daily was ineffective, and resulted in the recurrence of OPN after a month.…”

Granulomatosis with polyangiitis – therapeutic challenge

“…Rituximab is an effective remission-inducing agent in GPA. The addition of a conventional maintenance agent to RTX and GCs decreased the incidence of relapse and did not result in a higher incidence of adverse events [24]. Rituximab is generally well tolerated, but side effects include leucopenia and infection.…”