Abstract: Macrophage activation syndrome（MAS） is a severe，potentially life-threatening complication of rheumatic diseases. Early identification and intensive treatment are the keys to successful treatment. However，because of the lack of effective clinical controlled trial studies，there is no unified treatment options for MAS at present. In this paper，by reviewing a large number of domestic and foreign literatures，we recommend that high-dose glucocorticoid（GC） be used as the initial treatment for MAS. Continuing GC monotherapy for more than 2 weeks in patients with persistent high fever or MAS status is inappropriate. Combination therapy with cyclosporin A（CsA） and/or etoposide（VP16） is the first choice，and anakinra（abroad） is recommended as first-line drugs for MAS. Tocilizumab or ruxolitinib may be effective for refractory fever in the patients.

Key words: macrophage activation syndrome, rheumatic diseases, child

摘要： 巨噬细胞活化综合征（macrophage activation syndrome，MAS）是一种继发于风湿性疾病的具有潜在生命危险性的严重并发症，早期识别和强化治疗是成功救治的关键。然而，由于缺乏有效的临床对照试验研究，目前MAS尚无统一的治疗方案。该文综述国内外文献，推荐 MAS初始治疗首先大剂量糖皮质激素（glucocorticoid，GC）冲击；持续高热或MAS状态者，激素单药治疗不超过2周，首选联合环孢素A（cyclosporin A，CsA），或同时联合依托泊苷（etoposide，VP16），如仍有发热，必要时联合托珠单抗（tocilizumab，TCZ）或芦可替尼（ruxolitinib）；在某些地区，阿那白滞素（anakinra）已推荐为MAS一线治疗药物。

关键词: 巨噬细胞活化综合征, 风湿性疾病, 儿童