Juvenile idiopathic inflammatory myopathy: single center data

Purpose: Juvenile idiopathic inflammatory myopathies (JIIMs) are rare autoimmune diseases. Delay in treatment may cause serious morbidity and mortality. Aim of this study is to evaluate demographic and clinical features, laboratory data, treatment modalities and outcome of patients with JIIMs. Materials and Methods: 15 patients with JIIMs, followed between 2003 and 2017 at Pediatric Rheumatology Department were included this study. We retrospectively collected data from patients' medical records and analyzed them. Results: Out of the 15 patients, 13 (80 %) had Juvenile Dermatomyositis (JDM), 2 (13.3%) Juvenile Polymyositis (JPM) and 1 (6.7 %) Overlap Myositis (OM, with scleroderma). Clinical symptoms at diagnosis were proximal muscle weakness (100%), heliotrope rash and malar rash (86.7%), Gottron papule (73.3%). Early aggressive treatment with corticosteroid (100%) and methotrexate (86.7%) were started. Intravenous immunoglobulin in 4 patients (26.7 %), cyclosporine in 3 patients (20 %), cyclophosphamide in one patient (6.7 %), and mycophenolate mofetil in one patient (6.7%) were among additional immunosuppressive treatments. Eventually all patients achieved remission but two had calcinosis and one had lipodystrophy. Conclusion: Early diagnosis and early aggressive treatment are the key points for JIIMs. For this reason, international standard treatment and follow-up programs should be taken into consideration during the disease process.