川崎病并发冠状动脉病变的病因及其发病机制

Etiology and pathogenesis of coronary artery lesions in Kawasaki disease

川崎病是一种以全身中小血管炎为主要病理改变的急性发热性出疹性小儿疾病。该病的长期预后决定于其心血管并发症，尤其是冠状动脉病变（coronaryarterylesion，CAL）程度。川崎病并发的CAL临床治疗非常棘手。目前研究显示CAL的发生与超抗原、巨细胞病毒、RNA病毒等特殊病原体感染相关，一些共刺激分子、细胞因子、趋化因子、金属基质蛋白酶、三磷酸肌醇激酶等基因多态性也参与CAL发生；对川崎病动物模型及川崎病死亡患儿的冠状动脉研究发现，免疫系统异常活化、免疫细胞浸润冠状动脉致冠状动脉内皮细胞功能紊乱、细胞基质降解是CAL发病的关键步骤。该文就川崎病并发CAL的病因及其发病机制研究进展进行综述。

Kawasaki disease （KD）, also known as mucocutaneous lymph node syndrome, is an acute febrile rash illness with systemic small and medium vessel vasculitis in children. The long-term prognosis of KD is mainly determined by its cardiovascular complications, especially coronary artery lesion （CAL）. CAL is harm to children＇s physical and mental health. Studies have suggested that the incidence of CAL is associated with infection of superantigens,cytomegalovirus and RNA viruses. Gene polymorphisms of costimulatory molecules, cytokines, chemokines, matrix metalloproteinase, inositol triphosphate kinase are also involved in occurance of CAL. Abnormal activation of the immune system, immune cell infiltration in the coronary arteries, coronary endothelial dysfunction, extracellular matrix degradation are the key steps of CAL through KD animal models and coronary artery from children died from KD. This review aims to discuss progress in etiology and pathogenesis of CAL in KD.