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Twenty one patients with severe pemphigus who didn't respond to treatment with corticosteroids or in whom treatment with corticosteroids was contraindicated were given four weekly infusions of rituximab—a monoclonal antibody directed against the CD20 antigen of B lymphocytes. The drug had been shown to be effective against several other autoimmune diseases and pemphigus, but only in combination with intravenous immunoglobulin and when given in multiple cycles.
At three months, 18 out of 21 people had a complete remission, defined as the epithelialisation of all skin and mucosal lesions. Of these, 12 people had pemphigus vulgaris and six had pemphigus foliaceus. Two more patients had a complete remission at 180 or 360 days of follow-up, leaving only one patient with pemphigus foliaceus not responding to treatment with rituximab. At three years of follow-up, 18 patients were still in full remission, with eight of them not receiving any treatment with corticosteroids.
Two patients had serious side effects. One developed pyelonephritis one year after the treatment, and another died of septicaemia a year and a half after treatment with rituximab. Other side effects of rituximab, previously reported in the literature, include death from pneumocystis infection, toxic epidermal necrolysis, and progressive multifocal leukoencephalopathy.