Relevance of rituximab therapy in pemphigus vulgaris: analysis of current data and the immunologic basis for its observed responses

Abstract

Treatment of pemphigus vulgaris (PV) patients with rituximab therapy has not been critically evaluated. This article will provide in significant detail the available data to date, in order to provide a clinical and immunologic basis for clinicians to decide how best to treat recalcitrant PV patients with rituximab. PV is an autoimmune blistering disorder that affects the skin and mucous membranes. The immunopathology is well characterized, including the target antigens. PV patients have traditionally been treated with systemic corticosteroids and adjuvant immunosuppressive therapies. Clinical remission has been achieved in roughly 30% of patients. However, many patients experience severe side effects from this immunosuppression, including death. B-cell depletion therapy with rituximab therapy has been used to treat several autoimmune diseases including PV. In this article, we examined the data on 153 patients with PV who have been treated with rituximab. Our focus is on the clinical response of the patients with emphasis on adjuvant therapies, dosing regimens, potential adverse events and mechanism of action related to B-cell modulation during therapy. Importantly, the use of rituximab has increased clinical remission rates to 65% including many patients who were able to discontinue all systemic medications. Finally, an expert commentary is provided, which includes suggestions for optimizing current therapy and recommends the future direction of the field. The authors strongly endorse the use of rituximab in treatment of PV patients, particularly those nonresponsive to or who develop serious side effects to conventional therapy. Proper monitoring of patients including peripheral B-cell counts and overt signs of infection are warranted, given the potential for prolonged B-cell depletion.