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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 …
GE and GMR contributed equally.
Contributors GE, GMR and AV designed the study, collected data, analysed results and wrote the manuscript. MG performed statistical analysis. MLU, ES and DP contributed to clinical data and analysed the results. All authors revised the manuscript critically for important intellectual content and approved the final version.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.