Department of Rheumatology and Clinical Immunology, Amrita Institute of Medical Sciences and Research Centre, Ponekkara, Kochi, Kerala, 682 041, India.
Indian J Pediatr. 2024 Oct;91(10):1041-1048. doi: 10.1007/s12098-023-04896-z. Epub 2023 Nov 3.
Idiopathic inflammatory myopathies (IIMs) are a diverse group of diseases characterized by proximal muscle weakness and inflammation in skeletal muscle. Phenotypically, the subtypes include dermatomyositis, polymyositis, inclusion body myositis, and amyopathic dermatomyositis. The most common IIM in children is juvenile dermatomyositis (JDM). In contrast to adult dermatomyositis (DM), children are likely to have frequent relapses, vasculopathy, and long-term metabolic and other complications like lipodystrophy, insulin resistance, and calcinosis. Significant advances in our understanding of pathogenesis, disease course, and treatment of JDM has changed the therapeutic landscape and improved outcomes in children. Myositis-specific autoantibodies and myositis-associated autoantibodies have unique clinical associations, disease course and help predict response to therapy. A multidisciplinary approach including exercise programs and psychosocial support is essential. The first line of treatment is a combination of corticosteroids and methotrexate (MTX). Other targeted immunosuppressive therapy is used in refractory cases. Early recognition and timely referral to a specialist center remain pivotal to improving the mortality and morbidity associated with this disease.
特发性炎性肌病（IIM）是一组以骨骼肌炎症和近端肌无力为特征的多种疾病。表型上，亚类包括皮肌炎、多发性肌炎、包涵体肌炎和无肌病性皮肌炎。儿童中最常见的 IIM 是幼年皮肌炎（JDM）。与成人皮肌炎（DM）相比，儿童更有可能频繁复发、血管病变以及长期代谢和其他并发症，如脂肪营养不良、胰岛素抵抗和钙沉积症。我们对 JDM 的发病机制、疾病过程和治疗的理解的显著进展改变了治疗格局，改善了儿童的预后。肌炎特异性自身抗体和肌炎相关自身抗体具有独特的临床关联、疾病过程，并有助于预测对治疗的反应。多学科方法包括运动计划和社会心理支持是必不可少的。一线治疗是皮质类固醇和甲氨蝶呤（MTX）的联合治疗。在难治性病例中使用其他靶向免疫抑制治疗。早期识别和及时转介到专业中心仍然是改善与该疾病相关的死亡率和发病率的关键。
