A Controlled Trial of High-Dose Intravenous Immune Globulin Infusions as Treatment for Dermatomyositis

TLDR: High-dose intravenous immune globulin is a safe and effective treatment for refractory dermatomyositis and changes in immune-mediated muscle abnormalities were determined by repeated muscle biopsies.

Abstract: Background Dermatomyositis is a clinically distinct myopathy characterized by rash and a complement-mediated microangiopathy that results in the destruction of muscle fibers. In some patients the condition becomes resistant to therapy and causes severe physical disabilities. Methods We conducted a double-blind, placebo-controlled study of 15 patients (age, 18 to 55 years) with biopsy-proved, treatment-resistant dermatomyositis. The patients continued to receive prednisone (mean daily dose, 25 mg) and were randomly assigned to receive one infusion of immune globulin (2 g per kilogram of body weight) or placebo per month for three months, with the option of crossing over to the alternative therapy for three more months. Clinical response was gauged by assessing muscle strength, neuromuscular symptoms, and changes in the rash. Changes in immune-mediated muscle abnormalities were determined by repeated muscle biopsies. Results The eight patients assigned to immune globulin had a significant improvement in sco...