Barron K S
Section of Pediatric Rheumatology, Baylor College of Medicine, Houston, Texas.
Rheum Dis Clin North Am. 1991 Nov;17(4):907-19.
Kawasaki disease is an immunologically mediated diffuse vasculitis of childhood of unknown etiology. While most of the clinical features--including diffuse mucosal inflammation, indurative edema, rash, and lymphadenopathy--are self-limiting, coronary artery aneurysms and the possibility of thrombotic occlusion occurs in up to 20% of children. The epidemiologic and clinical features of this disease suggest an infectious etiology; however, a specific organism has not been consistently identified. An abnormal immune response to this as yet to be defined organism plays a critical role in the progression of this disease. The morbidity and mortality of this disease are related primarily to the potential cardiovascular complications. The natural history of the coronary artery aneurysms is that most lesions regress with time. Factors leading to a higher probability of regression include age less than 1 year, female sex, fusiform aneurysm, and maximum diameter less than 4 mm. Current recommendations for therapy include aspirin and IVIG. The range of dosages regimens for each medication are discussed in the text.
川崎病是一种病因不明的儿童免疫介导性弥漫性血管炎。虽然大多数临床特征——包括弥漫性黏膜炎症、硬结性水肿、皮疹和淋巴结病——都是自限性的，但高达20%的儿童会出现冠状动脉瘤和血栓性闭塞的可能性。这种疾病的流行病学和临床特征提示其病因具有传染性；然而，尚未始终如一地确定具体的病原体。对这种尚未明确的病原体的异常免疫反应在该疾病的进展中起关键作用。这种疾病的发病率和死亡率主要与潜在的心血管并发症有关。冠状动脉瘤的自然病程是大多数病变会随时间消退。导致消退可能性较高的因素包括年龄小于1岁、女性、梭形动脉瘤以及最大直径小于4毫米。目前的治疗建议包括使用阿司匹林和静脉注射免疫球蛋白。每种药物的剂量方案范围在文中进行了讨论。
