巨噬细胞活化综合征是川崎病一种罕见的并发症，因其有起病隐匿、进展快、影响全身多器官系统、致死率 高等特点，需要给予更多的重视。由于目前国际上暂无统一的川崎病合并巨噬细胞活化综合征的诊断标准和治疗方案，因 此常被漏诊或误诊。文章就川崎病并发巨噬细胞活化综合征的流行病学、免疫学机制、诊断和治疗等研究进展进行综述。

Macrophage activation syndrome (MAS) is a rare complication of Kawasaki disease and more attention is needed for it in clinic because of its insidious onset, rapid progression, multiple organ system involvement and high mortality. There is no international unified diagnostic criteria and treatment regimen for Kawasaki disease complicated with macrophage activation syndrome at present, so it is often missed or misdiagnosed. This article reviews the epidemiology, immunological mechanism, diagnosis and treatment of Kawasaki disease complicated with macrophage activation syndrome.