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Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with a frequently relapsing/refractory course.1 2 Rituximab (RTX) is an established treatment in the other ANCA-associated vasculitides, but data on EGPA are scarce.3 4 Mohammad5 reported that RTX can be effective in EGPA, particularly in ANCA-positive patients.
We report the outcomes of 21 patients with EGPA who received RTX-induction (1 g 2 weeks apart, on top of ongoing immunosuppression) for refractory/relapsing disease in 2011–2017 (table 1). Starting from 2014, we scheduled RTX-maintenance (500 mg/6 months)6 7 in patients responding to RTX-induction. Nine patients received RTX-maintenance, the others received RTX only for relapses (a single, 1g infusion). Complete remission was defined as Birmingham Vasculitis Activity Score (BVAS)=0 and prednisone ≤7.5 mg/day,3 partial remission as >50% BVAS reduction and prednisone ≤15 mg/day. Refractory disease was defined as failure to achieve remission, relapse as the recurrence/new onset of clinical manifestations requiring intensification of immunosuppression or increase in glucocorticoid dose >50%.8 All patients signed informed consent; the study …
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