Since 1990 we have treated all children newly diagnosed(8) or with exacerbation(2) of dermatomyositis with intravenous immunoglobulin (IVIG; 2 grams/kg every 2-4 weeks). All new cases also received daily prednisone and 4, with severe disease, required the addition of cyclosporine during the first month after diagnosis.

In all but 2 children, daily prednisone could be tapered to an alternate day regimen within 2 to 8 weeks after diagnosis; none of these patients has required daily steroids again. In 2 children attempts at withdrawing IVIG, without other changes, resulted in exacerbations and reinstitution of IVIG restored complete control of disease. In 2 other children exacerbations, at later stages of disease, were successfully managed with IVIG alone.

In severe disease, use of IVIG sometimes provided the difference between control and inadequate control.

Treatment failures included 1 patient where IVIG had no beneficial effect in controlling the disease; 1 child in whom intravenous access was a problem and methotrexate was preferred for an exacerbation; and 1 exacerbation in which occasional intravenous steroid pulses provided more effective control and replaced gamma globulin.

A multicenter controlled study is required to fully evaluate efficacy of IVIG in the initial treatment of juvenile dermatomyositis and in exacerbation of disease.