Cimaz Rolando, Falcini Fernanda
Department of Pediatrics, ICP, Milano, Italy.
Autoimmun Rev. 2003 Sep;2(5):258-63. doi: 10.1016/s1568-9972(03)00032-6.
Kawasaki disease (KD) is a febrile systemic vasculitis complicated by coronary and peripheral arterial aneurysms in 20-35% of untreated patients. It is reported as the commonest cause of acquired heart disease in children in developed countries, and may be a risk for adult ischaemic heart disease. Although KD has been reported all over the world, it is overexpressed among Asian populations, especially Japanese. The disease pathogenesis is still unknown and several theories have been proposed, including the possibility of an infection by a toxin-secreting microorganism and of a superantigen-driven process. Despite numerous efforts there is still no diagnostic test available for KD, and the diagnosis is based on clinical criteria after the exclusion of other diseases presenting with high persistent fever. Prompt diagnosis is critical, since the early administration of intravenous immunoglobulins and aspirin reduces the rate of coronary abnormalities to less than 5% of patients.
川崎病（KD）是一种发热性全身性血管炎，在20% - 35%未经治疗的患者中会并发冠状动脉和外周动脉瘤。据报道，在发达国家，它是儿童后天性心脏病最常见的病因，并且可能是成人缺血性心脏病的一个危险因素。尽管川崎病在世界各地均有报道，但在亚洲人群中，尤其是日本人中更为常见。该病的发病机制仍然不明，已经提出了几种理论，包括由分泌毒素的微生物感染以及超抗原驱动过程的可能性。尽管进行了大量努力，但仍然没有可用于川崎病的诊断测试，并且诊断是在排除其他伴有持续高热的疾病后基于临床标准进行的。及时诊断至关重要，因为早期静脉注射免疫球蛋白和阿司匹林可将冠状动脉异常的发生率降低至不到5%的患者。
