Clinical Outcomes in Juvenile Dermatomyositis

A. V. Ramanan, MBBS, MRCP, MRCPCH, B. M. Feldman, MD, MSc, FRCPC

Disclosures

Curr Opin Rheumatol. 2002;14(6) 

In This Article

Abstract and Introduction

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.

Juvenile dermatomyositis (JDM) is a multisystem-likely autoimmune-disease of unknown etiology that results from inflammation of the small vessels of the muscle, skin, gastrointestinal (GI) tract, and other organs. It has an incidence of 2-3 per 1,000,000 per year.[1,2,3,4] In the 1950s and 1960s, outcomes for JDM were poor.[5] Almost one-third of the patients died and one-third developed permanent, severe, physical limitations. However, the 1970s and 1980s have seen a marked decrease in mortality (now < 10%) with an improvement in functional outcomes.[6,7,8]

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