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

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