川崎病(Kawasaki diseases,KD)是一种主要发生在5岁以下婴幼儿的急性发热出疹性疾病,属急性自限性血管炎综合征,其发病率有逐年增高趋势,在发达国家或地区,KD所致的冠状动脉病变(coronary artery lesions,CAL)已取代风湿热成为小儿最常见的后天性心脏病,并且可能成为成年后缺血性心脏病的危险因素之一。KD 病因及免疫发病机制仍未完全清楚,大量流行病学及临床观察提示 KD 可能是感染因素所致的急性免疫调节紊乱,遗传因素亦与 KD 发病相关。已报道多种细菌、病毒、支原体及其代谢产物(如链球菌和葡萄球菌超抗原)等与 KD 发病有关,但目前仍未检测到致 KD 的单一病原微生物。感染导致免疫活性细胞(如 T细胞、单核/巨噬细胞)异常活化,所产生的细胞因子可能参与血管内皮损伤及干扰自身免疫耐受。急性自限性血管炎 Kawasaki disease (KD) is an acute febrile rash disease mainly occurring in infants under 5 years of age. It is an acute self-limiting vasculitis syndrome and its incidence has been increasing year by year. In developed countries or regions Coronary artery lesions (CALs) caused by KD have replaced rheumatic fever as the most common acquired heart disease in children and may be one of the risk factors for adult ischemic heart disease. Etiology and pathogenesis of KD is still not fully understood. A large number of epidemiological and clinical observations suggest that KD may be an acute immunological disorder caused by infection, and genetic factors are also associated with the pathogenesis of KD. A variety of bacteria, viruses, mycoplasmas and their metabolites (such as streptococcus and staphylococcal superantigen) have been reported to be involved in the pathogenesis of KD. However, no single pathogenic microorganism causing KD has been detected so far. Infection leads to abnormal activation of immunocompetent cells (such as T cells, monocytes / macrophages). The resulting cytokines may be involved in vascular endothelial injury and interfere with autoimmune tolerance. Acute self-limiting vasculitis