The lung is involved in juvenile dermatomyositis.

摘要: Background Juvenile dermatomyositis (JDM) is the main cause of chronic idiopathic inflammatory myopathy autoimmune origin in children. The aim this multicenter prospective study was to describe respiratory status and treatment children followed for JDM. Methods patients Clinical manifestations, pulmonary function tests (PFT), chest high-resolution computed tomography (HRCT) scan results, treatments their adverse effects were analyzed JDM. Results Twenty-one patients (median age: 9.9 years; range: 20 months–18 years) included. median disease duration at time analysis 3 years (range: 6 months–9 4 months). Overall 16 (76%) 21 presented with a involvement related JDM including interstitial lung (n = 3) and/or muscle (n = 7). Seven other nonspecific manifestations. Three had aspiration pneumonia. A HRCT performed 15 children, abnormalities observed 12. PFT patients. showed functional abnormalities: restrictive ventilatory defect or obstructive (n = 4). Six abnormal tests, three one an defect. One child acute pneumonia clearly but too young perform confirm diagnosis. Treatment comprised systemic corticosteroid all adjuvant immunosuppressive therapy 11. Adverse linked reported eight patients. Conclusion The frequency justifies systematic assessment measures strength. We suggest that indicated cases symptoms abnormalities. Longitudinal studies are needed assess pediatric characteristics, long-term outcomes, responses taking into account risk–benefit ratio. Pediatr Pulmonol. 2013; 48:1016–1025. © 2012 Wiley Periodicals, Inc.