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川崎病(Kawasaki disease,KD)是一种自身免疫性血管炎综合征,好发于5岁以下小儿,其最大的危害是引起冠状动脉病变,包括冠状动脉扩张、冠状动脉瘤等,严重者可发生心肌梗死.在川崎病急性期,大剂量静脉注射丙种球蛋白(intravenous immunoglobulin,IVIG)联合阿司匹林为标准治疗方案,可以有效地治疗川崎病,但仍有10％ ～ 20％的患者对初次IVIG治疗产生抵抗,这部分患者发生冠状动脉损害的风险增加.对IVIG无反应性KD需要额外治疗,一些新的治疗方案不断出现,如再次应用IVIG、糖皮质激素、IL-1受体拮抗剂等.在川崎病恢复期,以抗凝、预防血栓治疗为主,对于冠状动脉病变进展严重者,可酌情予以外科手术或介入治疗.该文针对川崎病治疗研究进展进行综述.“,”Kawasaki disease is an autoimmune vascufitis syndrome that occurs in children under 5 years of age.Its greatest harm is coronary artery disease,including coronary artery dilatation,coronary artery aneurysm and so forth.Moreover,myocardial infarction may occur in severe cases.In the acute phase of Kawasaki disease,high-dose intravenous immunoglobulin(IVIG) combined with aspirin can be used as the standard therapy for the treatment of Kawasaki disease,but 10％ to 20％ of the patients still have resistance to the initial IVIG treatment.There is an increased risk of coronary artery damage in these patients.Additional treatment is required for IVIG non-reactivity Kawasaki disease,and some new therapies are emerging,such as repeated use of IVIG,glucocorticoid and IL-1 receptor antagonists,etc.In the recovery period of Kawasaki disease,anticoagulant and prophylaxis therapy are the main choices,and surgical or interventional therapy may be performed as appropriate for the patients with severe coronary artery disease.This article reviews the research progress in the treatment of Kawasaki disease.