Abstract

Juvenile dermatomyositis is difficult to treat, compounded by complications of the disease itself as well as side effects of treatment. The mainstay of pharmacological management is corticosteroids, to which the disease is usually very responsive, but steroids have well established short and long term side effects. Refractory cases may be resistant to steroids or patients may become dependent on high doses, with relapse in clinical disease precipitated by reduction. Overtreatment with steroids and too rapid a reduction are common errors. Various second line agents have been used with success in complex or refractory cases, including cyclophosphamide, methotrexate, and cyclosporin. Intravenous immunoglobulin has been tried in the management of autoimmune diseases, either as an alternative to cytotoxic treatment or as a steroid sparing agent. The reported benefit in a number of childhood illnesses, including juvenile dermatomyositis, prompted this clinical trial in some of our more refractory cases. Over the past four years, nine children attending a dermatomyositis clinic have been treated with intravenous immunoglobulin. The two main indications were failure of conventional treatment and severe side effects from previous treatment, principally corticosteroids. All nine were still on treatment with various permutations of prednisolone, azathioprine, and cyclosporin. All nine showed clinical improvement at some point in their treatment with intravenous immunoglobulin. Of eight children on concurrent prednisolone, the dose could be reduced in six and kept the same in two.