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Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis

Authors
Clare Hutchinson, MDCM, FRCPC
Brian M Feldman, MD, MSc, FRCPC
Section Editors
Thomas JA Lehman, MD
Marc C Patterson, MD, FRACP
Deputy Editor
Elizabeth TePas, MD, MS

INTRODUCTION

Juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) are autoimmune myopathies of childhood. JDM is primarily a capillary vasculopathy, whereas JPM involves direct T cell invasion of muscle fibers similar to that seen in adult polymyositis [1-3]. However, as the diagnostic tools become more sophisticated (eg, biopsies that demonstrate inclusion body myositis or inflammatory dystrophies, or autoantibodies that are markers of particular types of myositis such as anti-signal recognition particle [SRP] and necrotizing myopathy), fewer patients are diagnosed with JPM, calling into question whether JPM is a specific entity. (See "Clinical manifestations of dermatomyositis and polymyositis in adults".)

The treatment, complications, natural history, and prognosis of JDM and JPM are reviewed here. The pathogenesis, clinical manifestations, and diagnosis of these disorders are discussed elsewhere. (See "Juvenile dermatomyositis and polymyositis: Epidemiology, pathogenesis, and clinical manifestations" and "Juvenile dermatomyositis and polymyositis: Diagnosis".)

OUR APPROACH

There are few data from randomized, controlled trials to guide management decisions in the children with JDM or JPM. Until better data exist, we use the approach discussed below, based upon observational studies and our clinical experience. The goals of treatment include control of the underlying inflammatory myositis and prevention and/or treatment of complications (eg, contractures and calcinosis).

Initial treatment — The intensity of initial therapy increases with increasing severity of the symptoms [4], which range from mild disease with muscle weakness and cutaneous manifestations to serious, life-threatening weakness, internal organ damage, and ulcerative skin lesions. In a 2008 survey of North American pediatric rheumatologists, the majority of respondents used a combination of glucocorticoids and methotrexate to treat children with JDM [5]. However, the dose, route of administration, and the use of adjunctive therapy (eg, intravenous immunoglobulin [IVIG] and hydroxychloroquine) varied. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) has proposed several treatment strategies for initial therapy for JDM based upon a North American survey of practice [6]. Our approach is consistent with the CARRA treatment strategies.

Mild to moderate disease — The initial treatment used for the majority of children with JDM or JPM is a combination of high-dose oral prednisone (2 mg/kg per day, maximum 80 mg/day, in divided doses given twice daily) and methotrexate (15 mg/m2, maximum 25 mg/dose once weekly, administered as a subcutaneous injection). Children who receive methotrexate should also be given folic acid at 1 mg per day to limit methotrexate toxicity.

                              

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Literature review current through: Nov 2016. | This topic last updated: Wed Jun 22 00:00:00 GMT+00:00 2016.
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