川崎病(Kawasaki Disease,KD)是一种原因不明的儿童常见的免疫相关性血管炎综合症,主要累及全身中小血管。因其侵犯冠状动脉,引起冠状动脉扩张和冠状动脉瘤。目前在欧美一些发达国家该病发病率已经超过风湿热,成为儿童期后天性心脏病的首位病因。川崎病的病因、发病机制目前仍不十分清楚,多种学说的深入研究,已取得了广泛的进展。免疫系统的异常活化及免疫损伤性血管炎是川崎病的显著特征,T细胞介导的异常免疫应答及细胞因子的级联放大效应导致血管内皮功能障碍是川崎病血管炎性损伤的基础。川崎病的诊断目前尚无特异的实验室诊断指标,主要根据临床表现做出诊断,经过修订的诊断标准可提高早期诊断率,心脏彩超检查对不完全KD的诊断起重要作用,但仍缺乏更为敏感的特征性诊断指标,不完全KD的早期诊断方法今后仍是儿科领域关注的热点问题。 Kawasaki Disease (KD) is a common immune-related vasculitis syndrome in children of unknown etiology and mainly involves the systemic small and medium blood vessels. Because of its violation of the coronary arteries, causing coronary artery dilatation and coronary aneurysms. Currently in some developed countries in Europe and America the incidence of the disease has exceeded rheumatic fever, become the first cause of acquired heart disease in childhood. The etiology and pathogenesis of Kawasaki disease are still not very clear at present. In-depth study of various theories has made extensive progress. Abnormal activation of the immune system and immune damage vasculitis is a significant feature of Kawasaki disease, T cell-mediated abnormal immune response and cascade of cytokine magnification leading to vascular endothelial dysfunction is Kawasaki disease vascular injury basis. Kawasaki disease diagnosis is currently no specific laboratory diagnostic criteria, mainly based on clinical diagnosis, made a revised diagnostic criteria can improve the early diagnosis rate, cardiac ultrasonography for incomplete KD diagnosis plays an important role, but still lacking more As a sensitive characteristic diagnostic indicator, the early diagnosis of incomplete KD is still a hot issue in pediatrics.