Elizabeth Stringer1, Brian M Feldman. 1. Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada.
Abstract
PURPOSE OF REVIEW: Juvenile dermatomyositis is a rare chronic inflammatory disease that primarily affects the muscles and skin. Immunosuppressive therapy has played a very important role in reducing mortality rates and morbidity. The review focuses on the spectrum of medications currently used in the treatment of juvenile dermatomyositis, highlighting new advances and unanswered questions. RECENT FINDINGS: Data regarding the treatment of juvenile dermatomyositis come almost entirely from retrospective studies with relatively small numbers of patients. Corticosteroids continue to be the accepted first-line therapy. Evidence that the addition of methotrexate at initiation of treatment allows corticosteroids to be tapered more rapidly with good outcomes exists. High-risk, refractory patients may benefit from intravenous cyclophosphamide. Results in refractory patients treated with rituximab are also encouraging. Topical immunosuppressant agents have been largely disappointing in treating rash. The effect and role of exercise in the treatment and rehabilitation of patients with juvenile dermatomyositis is an interesting new area of research. SUMMARY: Future research in the treatment of juvenile dermatomyositis should focus on improving the understanding of disease course and its predictors such that treatment protocols can be developed to provide the most benefit and least amount of medication toxicity for the individual patient.
PURPOSE OF REVIEW: Juvenile dermatomyositis is a rare chronic inflammatory disease that primarily affects the muscles and skin. Immunosuppressive therapy has played a very important role in reducing mortality rates and morbidity. The review focuses on the spectrum of medications currently used in the treatment of juvenile dermatomyositis, highlighting new advances and unanswered questions. RECENT FINDINGS: Data regarding the treatment of juvenile dermatomyositis come almost entirely from retrospective studies with relatively small numbers of patients. Corticosteroids continue to be the accepted first-line therapy. Evidence that the addition of methotrexate at initiation of treatment allows corticosteroids to be tapered more rapidly with good outcomes exists. High-risk, refractory patients may benefit from intravenous cyclophosphamide. Results in refractory patients treated with rituximab are also encouraging. Topical immunosuppressant agents have been largely disappointing in treating rash. The effect and role of exercise in the treatment and rehabilitation of patients with juvenile dermatomyositis is an interesting new area of research. SUMMARY: Future research in the treatment of juvenile dermatomyositis should focus on improving the understanding of disease course and its predictors such that treatment protocols can be developed to provide the most benefit and least amount of medication toxicity for the individual patient.