Improved clinical control of a challenging case of Behçet's disease with rituximab therapy

Behçet's disease (BD) is a rare form of vasculitis in North America. Like other rare diseases, there is no high-quality evidence for biologic use beyond case reports/series, and thus, therapy remains controversial. We report on the case of a patient who failed to respond to conventional therapy with colchicine and NSAIDs. She responded to steroids but failed to tolerate steroid tapers despite azathioprine, methotrexate, infliximab, and etanercept and required chronic prednisone up to 20 mg daily due to recurrent severe mucosal ulcers with fever, erythema nodosum (EN), and arthritis. She received cyclophosphamide for another indication but even then failed steroid taper. After three courses of rituximab, she showed marked clinical improvement and was able to reduce prednisone to 8 mg and to return to school and work. A review of disease mechanisms and clinical literature is presented for those facing challenging cases where evidence is limited.