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Churg-Strauss Syndrome (CSS) is a small vessel systemic vasculitis, characterised by asthma, peripheral eosinophilia, neuropathy, pulmonary infiltrates, and sinus abnormalities.1 Conventional treatment with corticosteroids and cyclophosphamide2 controls disease activity; however, relapse is frequent and the treatment is toxic. Alternative treatments include plasma exchange,3 interferon alfa,4 and intravenous immunoglobulin.5 B cell depletion with rituximab has proved effective in autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus, and antineutrophil cytoplasmic antibody (ANCA) associated vasculitis.6 We present two cases of patients with refractory CSS who were successfully treated with rituximab.
A 37 year old woman (case 1) presented with an 8 month history of nasal congestion, hearing loss, lymphadenopathy, rash, breast inflammation, peripheral neuropathy, abdominal pain, malaise, and weight loss. Tachydysrhythmias with poor left ventricular function on echocardiogram suggested cardiac vasculitis. Bone …
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