中国实用儿科杂志

中国实用儿科杂志 ›› 2023, Vol. 38 ›› Issue (6): 450-455.DOI: 10.19538/j.ek2023060609

• 专题笔谈 • 上一篇    下一篇

全身型幼年特发性关节炎发病机制及精准诊治进展

  

  1. 重庆医科大学附属儿童医院风湿免疫科 儿童感染免疫重庆市重点实验室 儿童发育疾病研究教育部重点实验室 国家儿童健康与疾病临床医学研究中心 儿童发育重大疾病国家国际科技合作基地，重庆  400014
  • 出版日期:2023-06-06 发布日期:2023-07-20
  • 通讯作者: 唐雪梅，电子信箱：tangxuemei2008@163.com
  • 基金资助:
    “十四五”国家重点研发计划资助项目（编号2021YFC2702003）

Progress in pathogenesis and precise diagnosis and treatment of systemic juvenile idiopathic arthritis

  1. Department of Rheumatology and Immunology， Children’s Hospital of Chongqing Medical University，Chongqing Key Laboratory of Child Infection and Immunity， Children’s Hospital of Chongqing Medical University，Ministry of Education Key Laboratory of Child Development and Disorders， Children’s Hospital of Chongqing Medical University，National Clinical Research Center for Child Health and Disorders， Children’s Hospital of Chongqing Medical University. China International Science and Technology Cooperation Base of Child  and Critical Disorders. Chongqing400014， China
  • Online:2023-06-06 Published:2023-07-20

摘要： 全身型幼年特发性关节炎（systemic juvenile idiopathic arthritis，sJIA）是幼年特发性关节炎的一种亚型，具有独特的临床及病理特点。巨噬细胞活化综合征（macrophage activation syndrome，MAS）最常并发于sJIA，病情进展迅速，可危及生命。sJIA合并MAS的病因尚不明确，仍是目前儿童风湿病面临的巨大挑战。文章概述了sJIA诊治进展，强调疾病的早期预警、诊断及精准治疗，以降低致残致死率，改善疾病预后。

关键词: 全身型幼年特发性关节炎, 巨噬细胞活化综合征, 诊断, 治疗

Abstract: Systemic juvenile idiopathic arthritis （sJIA） is a subtype of juvenile idiopathic arthritis， which has unique clinical and pathological characteristics. Macrophage activation syndrome （MAS） most frequently occurs in sJIA. It progresses rapidly and can be life-threatening. The etiology of sJIA and sJIA with MAS remains unclear，which is a major challenge in childhood rheumatism. This review summarizes the progress in the diagnosis and treatment of sJIA， emphasizing the early warning，diagnosis and precise treatment， to reduce the rate of disability and mortality and improve the prognosis of the disease.

Key words: systemic juvenile idiopathic arthritis, macrophage activation syndrome, diagnosis, treatment