Clinical outcomes in juvenile dermatomyositis.

Juvenile dermatomyositis is a chronic inflammatory illness of unknown etiology that affects primarily muscle and skin. It has an incidence of 2-3 per 1,000,000, per year. The disease can affect other organ systems, including the gastrointestinal tract, lungs, and heart. In addition, calcinosis is seen in one-third of patients. The mainstay of therapy is corticosteroids; some children require additional immunosuppressive agents because of corticosteroid resistance or intolerance. Functional outcomes have become good with modern treatments, but the disease remains chronic in a large number of children and sequelae are often seen.