巨噬细胞活化综合征(macrophage activation syndrome，MAS)是川崎病(Kawasaki disease，KD)少见且致命的并发症。迄今为止，已经探究了许多可能的病因，包括感染因素及免疫机制等，但确切的病因仍不清楚。因其起病隐匿、进展迅速、影响全身多器官系统、临床症状危重及致死率较高等特点，MAS需要尽早诊断并及时给予合理的治疗。目前国际上尚缺乏KD合并MAS(KD-MAS)的规范诊断标准和治疗方案，因此许多患儿常被漏诊或误诊并错过最佳治疗时机。该文旨在从流行病学、发病机制、临床特点、诊断及治疗等方面详细分析KD-MAS，以期制定更具体的诊断标准及更规范的治疗方案，使此类重症KD患儿得到及时、准确的诊断并接受合理的治疗。“,”Macrophage activation syndrome(MAS)is a rare and fatal complication of Kawasaki disease.So far, many possible causative factors have been explored, including infectious agents and immunologic mechanisms, but the exact etiology remains unclear.Early diagnosis and timely and reasonable treatment are needed for it, because of its insidious onset, rapid progression, multiple organ system involvement, critical clinical symptoms and high mortality.There is no international unified diagnostic criteria and treatment regimen for Kawasaki disease complicated with macrophage activation syndrome at present, many children have the missed diagnosis and miss the best time for treatment.This review aims to analyze Kawasaki disease complicated with macrophage activation syndrome in detail in terms of epidemiology, pathogenesis, clinical characteristics, diagnosis and treatment in order to establish the more specific diagnostic criteria and the more standardized treatment to enable children with such severe KD to receive timely diagnosis and appropriate treatment.