中国实用儿科杂志

中国实用儿科杂志 ›› 2025, Vol. 40 ›› Issue (9): 724-728.DOI: 10.19538/j.ek2025090604

• 专题笔谈 • 上一篇    下一篇

对中国幼年皮肌炎诊断与治疗指南的再认识

  

  1. 深圳市儿童医院风湿免疫科，广东  深圳  518034
  • 出版日期:2025-09-06 发布日期:2025-10-20
  • 通讯作者: 杨军，电子信箱：rogasansz@163.com
  • 基金资助:
    国家重点研发计划项目（2021YFC2702001）；深圳市三名工程项目（SZSM202411012）

Renewed understanding of the diagnosis and treatment guidelines for juvenile dermatomyositis in China

  1. Department of Rheumatology and Immunology，Shenzhen Children’s Hospital，Shenzhen  518034，China
  • Online:2025-09-06 Published:2025-10-20

摘要： 幼年型皮肌炎（juvenile dermatomyositis，JDM）是一种以皮肤和肌肉炎症为主要临床特征的慢性自身免疫性疾病，需根据疾病严重程度及主要受累器官进行个体化治疗。糖皮质激素常规剂量联合甲氨蝶呤为非重症病例的一线治疗方案，甲泼尼龙冲击治疗适用于重症病例。近年研究逐渐证实了静脉注射免疫球蛋白（IVIG）对JDM的疗效。生物制剂及JAK抑制剂可选择性用于治疗难治性病例，嵌合抗原受体T细胞疗法（CAR-T）也存在一定治疗前景。

关键词: 幼年型皮肌炎, 生物制剂, JAK 抑制剂, 静脉注射免疫球蛋白

Abstract: Juvenile Dermatomyositis （JDM） is a chronic autoimmune disease primarily characterized by skin and muscle inflammation， requiring individualized treatment based on disease severity and major organ involved. For non-severe cases， a combination of conventional-dose glucocorticoids with methotrexate serves as the first-line therapy. Methylprednisolone pulse therapy is indicated for severe cases. Recent studies have progressively confirmed the efficacy of intravenous immunoglobulin （IVIG） in JDM. Biological agents and JAK inhibitors may be selectively used for refractory cases， while CAR-T therapy also holds therapeutic promise for these refractory patients.

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