Anti-CD20 monoclonal antibody (rituximab) in the treatment of pemphigus.

BACKGROUND Pemphigus is a severe autoimmune blistering disorder caused by autoantibodies to desmoglein 1 and 3. The disease course is typically severe, thus requiring multiple immunosuppressive agents. The treatment is still challenging and in some patients with recalcitrant disease, therapies fail and therapeutic options are limited. OBJECTIVES To investigate whether depletion of B lymphocytes that are thought to produce disease-causing autoantibodies shows a long-term benefit in pemphigus. METHODS Five patients diagnosed as having pemphigus vulgaris and pemphigus foliaceus were treated with the monoclonal antibody rituximab. Rituximab was administered intravenously at a dosage of 375 mg m(-2) once weekly for 4 weeks. RESULTS The treatment was well tolerated and all patients showed a good response over a follow-up period of up to 3 years, allowing immunosuppressive treatment to be reduced or terminated. B-cell depletion persisted for 6-12 months, and in one patient for almost 3 years. CONCLUSIONS This study highlights the prolonged effect and disease control after one single course of rituximab and further extends the spectrum of treatments of bullous autoimmune disorders.