Treatment of severe pemphigus with rituximab: report of 12 cases and a review of the literature.

摘要: Background Treatment of pemphigus vulgaris can be challenging. Systemic steroids associated with other immunosuppressant agents are the mainstay therapy and have dramatically reduced morbidity mortality from vulgaris. In some patients, however, these not able to control disease or severe adverse effects. Rituximab (MabThera; Roche, Basel, Switzerland), a chimeric monoclonal anti-CD20 antibody, induces depletion B cells in vivo has shown efficacy patients refractory antibody-mediated autoimmune disorders. We report 10 cases 2 foliaceous treated rituximab—to our knowledge largest series so far—and review existing literature on topic. Observation The 12 were selected for treatment antibody. was administered intravenously at dosage 375 mg/m once weekly 4 weeks. well tolerated, all showed good clinical response during an 18-month follow-up period, along consensual decline serum antidesmoglein titers. No infectious complications observed. Conclusions is induce prolonged remission both after single course treatments. preliminary experiences worldwide make rituximab promising therapeutic option diseases. high costs limited long-term effects, limit its use treatment-resistant life-threatening disease.