Annesofie Faurschou1, Robert Gniadecki. 1. Department of Dermatology, University of Copenhagen, Bispebjerg Hospital, Denmark. af16@bbh.hosp.dk
Abstract
BACKGROUND: Pemphigus vulgaris (PV) is a severe autoimmune blistering disease involving the skin and mucous membranes. The response to therapy varies greatly amongst patients and treatment may be challenging. Rituximab is a chimeric monoclonal antibody that selectively targets cell surface antigen CD20, thus depleting mature B cells in vivo. METHODS: We report the results of rituximab treatment in two patients with severe PV. In both patients, high-dose oral prednisolone and adjuvant therapy with intravenous immunoglobulins and mycophenolate mofetil failed to control disease activity. Consequently, the patients were treated with two courses of four weekly intravenous infusions of rituximab (375 mg/m(2)) with a 6-month interval. RESULTS: Clinical improvement was already noticeable 3-6 weeks after the first infusion. After the second course, complete remission was achieved. Oral prednisolone was reduced and treatment with mycophenolate mofetil was continued. The patients remained in full remission 6 months after the last rituximab infusion. CONCLUSION: These cases suggest that two courses rather than a single course of rituximab may be a preferable mode of treatment. Rituximab should be considered as a promising treatment option for recalcitrant PV.
BACKGROUND: Pemphigus vulgaris (PV) is a severe autoimmune blistering disease involving the skin and mucous membranes. The response to therapy varies greatly amongst patients and treatment may be challenging. Rituximab is a chimeric monoclonal antibody that selectively targets cell surface antigen CD20, thus depleting mature B cells in vivo. METHODS: We report the results of rituximab treatment in two patients with severe PV. In both patients, high-dose oral prednisolone and adjuvant therapy with intravenous immunoglobulins and mycophenolate mofetil failed to control disease activity. Consequently, the patients were treated with two courses of four weekly intravenous infusions of rituximab (375 mg/m(2)) with a 6-month interval. RESULTS: Clinical improvement was already noticeable 3-6 weeks after the first infusion. After the second course, complete remission was achieved. Oral prednisolone was reduced and treatment with mycophenolate mofetil was continued. The patients remained in full remission 6 months after the last rituximab infusion. CONCLUSION: These cases suggest that two courses rather than a single course of rituximab may be a preferable mode of treatment. Rituximab should be considered as a promising treatment option for recalcitrant PV.