Azathioprine in the treatment of pemphigus vulgaris. A long-term follow-up.

In a prospective long-term study, thirty-seven patients with severe generalized pemphigus vulgaris were treated with a combined corticosteroid-azathioprine regimen. Twenty-nine patients were available for complete follow-up lasting from 4 to 16 years after initiation of therapy. At the time of final evaluation, twenty-seven patients (93%) were alive; two deaths were unrelated to therapy; thirteen (45%) of the patients were free of disease and had not received treatment for up to 132 months; five of these patients had been off therapy for periods ranging from 60 to 132 months; eleven (38%) of the patients were clinically free of disease but still had low titers of antibodies and thus required low-dose maintenance therapy; five (17%) of the patients were well controlled but not completely free of disease. Side effects were rare and mostly related to corticosteroids. Of the original thirty-seven patients, only one death related to disease or therapy occurred and was due to pulmonary tuberculosis. It is concluded that azathioprine-corticosteroid treatment of pemphigus is highly effective and safe; it leads to long-term remissions in most patients and possibly to a cure in some.