摘要： 皮肌炎的临床特征是皮肤改变和骨骼肌炎症反应，亦可累及其他器官。发病机制包括易感基因、环境应激源、免疫和非免疫诱发机制。临床主要表现为亚急性进行性近端肌无力、皮疹或二者兼有，以及肌外表现，如心脏异常、间质性肺病和恶性肿瘤。辅助检查包括肌炎自身抗体、肌酶谱、肌电图。针对皮肤损害，予以抑制光敏感药物和抗疟药；合并肌肉受累时系统性激素治疗是一线方案；免疫抑制剂常用于难治性皮肌炎或激素出现不良反应时的添加治疗；其他治疗方法还包括钙调磷酸酶抑制药、环磷酰胺、生物制剂、Janus激酶抑制剂等。

关键词: 皮肌炎, 自身抗体, 肾上腺皮质激素类, 免疫抑制剂, 综述

Abstract: Dermatomyositis (DM) is characterized by skin lesions, skeletal muscle inflammation, and involvement of other organs. The pathogenesis of DM includes susceptibility genes, environmental stressors, immune and non-immune inducing mechanisms. DM usually presents as subacute progressive proximal muscle weakness, skin rash, or both. Other common extramuscular findings include various heart abnormalities, interstitial lung disease (ILD), and malignant tumors. The auxiliary examination of DM includes the examination of myositis autoantibodies, myoenzymes and electromyography (EMG). Skin treatments for DM include photosensitivity inhibition drugs and antimalarial drugs. If the DM is associated with muscle involvement, systemic corticosteroids is the first line of treatment, and immunosuppressants are used for refractory diseases or as corticosteroids sparing agents in the event of side effects. Other treatments include oral calcineurin inhibitors, cyclophosphamide, biologic agents and Janus kinase (JAK) inhibitor.

Key words: Dermatomyositis, Autoantibodies, Adrenal cortex hormones, Immunosuppressive agents, Review