Juvenile Dermatomyositis in Iranian Children; a Case Series Report

Abstract

Objective: Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children, distinguished by proximal muscle weakness and a characteristic rash. Inflammatory cell infiltrates result in vascular inflammation, the underlying pathology in this disorder. Extramuscular manifestations include joint contracture, dysphagia, cardiac disturbances, pulmonary symptom and subcutaneous calcifications. This study is aimed to evaluate the clinical features and outcomes of juvenile dermatomyositis (JDM) in Iranian children and compare the achieved data with the relative literature. Methods: We retrospectively studied 14 patients, whose diagnosis of juvenile dermatomyositis was made according to the Bohan and Peter\\\'s diagnostic criteria. During the last 7 years we evaluated the clinical manifestations and the outcomes of these patients. Results: Average onset age was 7 years. Muscle weakness occurred in 85%; heliotrope rash 57%; Gottron papules 35%, calcinosis 14.2%; 74.9% had at least one abnormal muscle enzyme result. Muscle biopsy performed in 31.8% was abnormal in 90.9% of cases and electromyogram was abnormal in 85.7%. All patients received corticosteroids; some patients had in addition at least one as needed: methotrexate, hydroxychloroquine, IV immunoglobulin, or cyclosporin. Mean follow up of these patients was 36 months and one case of mortality happened in follow up period. Conclusion: This study indicates that early diagnosis and aggressive treatment is highly successful in minimizing the long term sequelae of JDM, including calcinosis.