川崎病治疗的研究进展

Research Progress in the Treatment of Kawasaki Disease

摘要: 川崎病又称皮肤粘膜淋巴结综合征，是一种多系统血管炎症性疾病，发病主要见于婴幼儿及5岁以下儿童。流行病学研究表明病原微生物侵入遗传易感儿童，导致其自身免疫功能紊乱而引起KD发病。在KD急性期，免疫系统过度激活，导致内皮功能障碍和受损的血管壁重塑，从而引起冠状动脉扩张、冠状动脉瘤及血栓形成等并发症。但其确切病因及发病机制不明。未接受规范化治疗的川崎病患儿约15%~25%会发展为冠状动脉病变，而早期接受静注人免疫球蛋白(Intravenous immunoglobulin, IVIG)和口服阿司匹林(Aspirin, ASP)治疗可将冠状动脉瘤的发生率降至5%以下，但仍有部分川崎病患儿可形成冠状动脉病变，有必要为患有川崎病的儿童制定额外的治疗策略。针对发病机制的不同步骤可以为我们提供替代治疗方案。

Abstract: Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a multi-system vascular inflammatory disease, mainly in infants and children under 5 years old. Epidemiological studies have shown that pathogenic microorganisms invade genetically susceptible children, leading to au-toimmune dysfunction and KD. In the acute phase of KD, the immune system is over activated, leading to endothelial dysfunction and impaired vascular wall remodeling, resulting in complica-tions such as coronary artery dilatation, coronary aneurysm and thrombosis. However, its exact eti-ology and pathogenesis are unknown. About 15% to 25% of children with Kawasaki disease who do not receive standardized treatment will develop coronary artery disease. Early intravenous immu-noglobulin (IVIG) and oral aspirin (ASP) treatment can reduce the incidence of coronary artery an-eurysm to less than 5%. However, some children with Kawasaki disease can develop coronary ar-tery disease, and it is necessary to develop additional treatment strategies for children with Kawa-saki disease. Different steps of the pathogenesis can provide us with alternative treatment options.