中国实用儿科杂志

中国实用儿科杂志

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儿童风湿病国际相关诊治指南系列解读之四——儿童风湿病合并巨噬细胞活化综合征诊治指南解读

  

  1. 国家儿童医学中心（上海） 复旦大学附属儿科医院风湿科，上海 201102
  • 出版日期:2020-04-06 发布日期:2020-04-20

Interpretation of international guidelines for diagnosis and treatment of rheumatism in children（Ⅳ）：Interpretation of diagnosis and treatment guideline for pediatric rheumatic diseases complicated with macrophage activation syndrome

  1. Department of Rheumatology，National Children’s Medical Center（Shanghai），Shanghai Children’s Hospital of Fudan University，Shanghai 201102，China
  • Online:2020-04-06 Published:2020-04-20

摘要： 巨噬细胞活化综合征（MAS）是一种继发性噬血细胞性淋巴组织细胞增多症，是儿童风湿性疾病常见的致命并发症。MAS最常见的原发病是全身型幼年特发性关节炎（SJIA），约10%的SJIA可并发MAS。早期诊断和快速启动免疫抑制是有效治疗MAS的关键。随着对MAS病理生理学的深入理解和新疗法的出现，广泛的免疫抑制治疗方法正在被“靶向抗细胞因子疗法”所取代。随着对儿童风湿性疾病中MAS早期识别意识的提高，结合有效、毒性较小的细胞因子“靶向治疗”的使用，应该可以降低这类致死性较高疾病的病死率。

关键词: 噬血细胞性淋巴组织细胞增多症, 巨噬细胞活化综合征, 诊断, 鉴别诊断, 炎症因子靶向治疗

Abstract: Macrophage activation syndrome（MAS） is a secondary hemophagocytic lymphohistiocytosis，which is a common and fatal complication of rheumatic diseases in children. The most common primary disease of MAS is systemic juvenile idiopathic arthritis（SJIA），about 10% of which can develop MAS. Early diagnosis and rapid initiation of immunosuppression are the key to the effective treatment for MAS. With the deep understanding of the pathophysiology of MAS and the emergence of new therapies，a wide range of immunosuppressive therapies are being replaced by targeted anti-cytokine therapies. With the improvement of early recognition awareness of MAS in children’s rheumatic diseases，and through combination with the use of effective and less toxic cytokine targeted therapy，the mortality of such deadly diseases can be reduced.

Key words: hemophagocytic lymphohistiocytosis, macrophage activation syndrome, diagnosis, differential diagnosis, cytokine-targeted therapies