Abstract

Dermatomyositis is an uncommon idiopathic chronic inflammatory disorder. Oral corticosteroids are the treatment of choice. A few patients become resistant to steroids. We describe two patients who, after failure to respond to oral and high-dose intravenous corticosteroids, received a combination of oral cyclosporine and high-dose intravenous immune globulin, with apparent remission. The first patient was a 6-year-old girl with recurrent disease and vasculitis, despite prednisone therapy and normal muscle enzyme levels. The response was remarkable within 3 weeks of therapy. The second patient was a 30-year-old woman with progressive disease and secondary respiratory failure despite oral prednisone and methotrexate therapy. The response to treatment was optimal within 4 weeks. She received maintenance low-dose cyclosporine and a tapering dose of prednisone. The combination of high-dose immune globulin and cyclosporine can be useful and safe in the management of steroid-resistant dermatomyositis.

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