Eosinophilic Granulomatosis With Polyangiitis and Cardiac Involvement: A Case Report

摘要

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is characterized by asthma, eosinophilia and small vessel vasculitis affecting the lungs, nasal sinuses, peripheral nerves, and heart [1]. Although antineutrophil cytoplasmic antibodies (ANCAs) play an important role in the pathogenesis of EGPA, many patients present the condition without these specific antibodies, especially patients with cardiac involvement [2]. Rituximab, a monoclonal anti-CD20 antibody, was recently included in the EULAR ANCA-associated vasculitis treatment recommendations [3]. Two published trials involving patients with granulomatosis with polyangiitis and with microscopic polyangiitis, respectively, showed rituximab to be safe and effective in inducing remission [4,5]. As far as EGPA is concerned, rituximab has been reported as a treatment option in very few case reports [6]. The potential pathogenic role of ANCAs supports the use of rituximab in EGPA, as well as B cell–dependent activation of T lymphocytes with subsequent IL-5 production. We report the case of a patient affected by ANCA-negative EGPA with life-threatening heart and pulmonary involvement who was successfully treated with rituximab.