Clinical features and outcomes of juvenile dermatomyositis and other childhood onset myositis syndromes

Childhood forms of myositis show less heterogeneity than myositis in adults. The majority of patients have juvenile dermatomyositis (JDM). The most common manifestations of JDM are skin rash (often consisting of a heliotrope eyelid discoloration and Gottron's papules) and proximal muscle weakness. Other organ systems, including the gastrointestinal tract, lungs, heart, articular, visual, and nervous system can be involved. Prognosis has improved with modern treatments, but the disease becomes chronic in a large number of children. The mainstay of therapy remains corticosteroids, however, we and others have recently introduced methotrexate early in the disease course in an effort to reduce the total cumulative dose and duration of steroid treatment. This article reviews our experience over the past decade in caring for a large population of children with myositis with the goal of describing the current clinical picture and outcomes given the more aggressive therapies in use today.