Pemphigus is a severe life-threatening blistering disease associated with autoantibodies against cell adhesion proteins desmogleins 1 and 3. Patients with severe pemphigus commonly show high rates of relapse after conventional immunosuppressive therapy. The newly developed drug Rituximab showed impressing promises in the treatment of refractory pemphigus vulgaris (PV). In the present study the efficacy of a single course rituximab therapy in the treatment of PV was investigated. Eighteen patients with severe recalcitrant PV were recruited to this study. Pemphigus disease activity index (PDAI), anti-desmoglein 1 and anti-desmoglein 3 antibody titers, and percent of CD20 positive cells were measured at baseline, 10 ± 1, and 22 ± 2 weeks after rituximab therapy. Rituximab was given intravenously at dose 375 mg/m(2) once weekly for 4 weeks. Rituximab therapy caused a dramatic reduction in the PDAI, accompanied by decreases in anti-desmoglein 1 and anti-desmoglein 3 antibody titers over the follow-up course. The B-cell population decreased at the first follow-up, but returned to its baseline levels at the second follow-up. Rituximab therapy decreased the dose of immunosuppressive drugs required to control the disease. It seems that the rituximab may be effective and safe for treatment of refractory PV.
Keywords: CD20 positive cells; Pemphigus vulgaris; Rituximab; anti-desmoglein 1 antibody; nti-desmoglein 3 antibody.