Intravenous immunoglobulin therapy for juvenile dermatomyositis: Efficacy and safety

Objective. To assess the efficacy of intravenous immunoglobulin (IVIG) for the treatment of juvenile dermatomyositis (JDM) in patients who were unresp onsive to corticosteroids (steroid resistant or steroid dependent) or showe d unacceptable toxicity. Methods. A retrospective chart review of the course of all patients with JD M treated with IVIG who attended the Dermatomyositis Clinic at The Hospital for Sick Children, Toronto, Canada, from August 1986 to December 1996. Results. Eighteen patients with JDM were treated with IVIG. Ten patients we re taking additional ?,nd line treatments, methotrexate, azathioprine, cycl osporine, and cyclophosphamide. The main indication for starting IVIG was t he failure of steroid therapy to induce remission of JDM. Twelve patients s howed clinical improvement with IVIG. In these patients, the corticosteroid dose was reduced by > 50% for > 3 months without clinical or biochemical f lare. Nine of these 12 patients had IVIG alone as a 2nd line agent, whereas 3 patients were treated with additional agents. Six patients remained ster oid dependent; they subsequently required multiple agents to induce remissi on of JDM. Conclusion. Most steroid dependent or steroid resistant patients in our cli nic were able to markedly reduce their dose of corticosteroid with the addi tion of IVIG. Given the retrospective nature of our data and the fact that multiple agents were sometimes used together, it will be important to confi rm these findings in a controlled trial.