Immunosuppressive agents in corticosteroid-refractory childhood dermatomyositis

Five patients with childhood dermatomyositis, followed for 18 to 96 months, improved when treated with a combination of corticosteroids and methotrexate (one patient) or corticosteroids and cyclophosphamide (four patients) after having become refractory to corticosteroid therapy alone. Complications of vascular involvement in childhood dermatomyositis or from immunosuppressive therapy were observed in most of these patients. Disease components involving skin, muscle, or systemic vessels sometimes varied independently in regard to disease activity or therapeutic responsiveness. In two patients with stable or improving muscle function, cerebrovascular complications occurred. Monitoring of disease activity was best accomplished by clinical evaluation; serum muscle enzymes usually failed to rise prior to or during periods of clinical worsening.