Dirençli pemfigus vulgaris olgularında ritüksimab tedavisinin etkinlik ve güvenirliği.

Background and Design: Pemphigus vulgaris (PV) is a severe, chronic, potentially life-threatening autoimmune blistering disease that affects the skin and mucous membranes, associated with the loss of cell-cell adhesion and blister formation. Systemic steroids in combination with immunosuppressive agents are the mainstay of therapy in pemphigus. Rituximab, a chimeric monoclonal anti-CD20 antibody, has been tried increasingly for the treatment of PV. Materials and Methods: We sought to test the efficacy and safety of rituximab as an adjuvant therapy by retrospective analysis of clinical and immunological data of patients. We performed a retrospective analysis of 13 patients with refractory pemphigus vulgaris who were treated with rituximab at Akdeniz University Hospital, Clinic of Dermatology and Venereology, Bullous Disease Unit. We evaluated clinical and immunological data with last treatments. Results: The patients were treated with one cycle of two biweekly infusions of rituximab at a dose of 1000 mg on days 1 and 15, except one received four doses of 375 mg/m2 weekly. The mean follow-up time was 18.5 months. All patients had a decrease in antibody titers or antibodies were completely undetected after treatment. Rituximab use resulted in a significant reduction in steroid dosage during follow-up. At the end of the follow-up period, 7 patients achieved complete disease remission without therapy, 1 patient achieved partial disease remission without therapy, 2 patients achieved complete remission on minimal therapy, 1 patient achieved complete remission on therapy, 1 patient achieved partial remission on minimal therapy, and one patient was lost to follow-up. Rituximab was well tolerated by all patients. Clinical relapse was seen in 7 patients (53.8%) in the mean period of 13.8 months. Relapses have been managed with additional infusions of rituximab. Conclusion: Rituximab is beneficial in the management of refractory PV, induces prolonged clinical remission and has a steroid-sparing effect. (Turkderm 2015; 49: 117-24) [ABSTRACT FROM AUTHOR]