Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome.

摘要

BACKGROUND: Treatments for Churg-Strauss syndrome (CSS), a rare eosinophilic vasculitis characterized by asthma, sinusitis, peripheral eosinophilia, pulmonary infiltrates, and tissue infiltration, are limited by toxicity or poor efficacy. Levels of IL-5, a cytokine regulating eosinophils, can be increased in patients with CSS. Mepolizumab, a humanized monoclonal anti-IL-5 antibody, decreases steroid requirements in patients with non-CSS hypereosinophilic syndromes. OBJECTIVE: The purpose of this study was to assess whether mepolizumab would safely allow corticosteroid tapering in patients with steroid-dependent CSS while decreasing serum markers of disease activity. METHODS: This open-label pilot study treated 7 patients with 4 monthly doses of mepolizumab to assess whether it safely decreased CSS disease activity and permitted tapering of systemic corticosteroids. RESULTS: Mepolizumab was safe and well tolerated in patients with CSS. Mepolizumab reduced eosinophil counts and allowed for safe corticosteroid reduction in all 7 subjects. On cessation of mepolizumab, CSS manifestations recurred, necessitating corticosteroid bursts. CONCLUSION: Mepolizumab is a safe and well-tolerated therapy in patients with CSS, offering clinical benefit by enabling corticosteroid tapering while maintaining clinical stability.