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Abstract

Background  Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Various treatment modalities have been described to treat pemphigus. In cases where the disease fails to respond to conventional therapy, rituximab has been shown to be effective.

Objective  To study the efficacy of rituximab in the treatment of resistant or severe pemphigus in Indian patients.

Methods  Patients with pemphigus were treated with intravenous rituximab 1000 mg in adults or 375 mg/m2 body surface area in children by two doses, 15 days apart in this open labelled pilot study. Anti-desmoglein1 (anti-Dsg1) antibodies and anti-desmoglein3 (anti-Dsg3) antibodies were measured at the start of therapy and at the end of the follow-up period. The outcome was studied in terms of control of disease activity (CD), complete remission (CR), partial remission (PR) and time to disease control (TDC) as defined by the consensus statement from the International Pemphigus Committee.

Results  A total of 9 (90%) of 10 patients responded to the treatment. Three (30%) had CR of disease and were off all treatment. Four (40%) patients had CR and were on low dose oral prednisolone. Two (20%) patients had PR and were on low dose prednisolone. One patient died of sepsis. The mean TDC was 8 weeks. Response to treatment showed good correlation with index values of anti-Dsg1 antibody. Infusion-related angioedema and sepsis were seen as complications due to rituximab administration.

Conclusion  Rituximab is effective in treating resistant and severe pemphigus in Indian patients. Acute complications can occur during rituximab infusion and require close monitoring.