Shulman Stanford T
Pediatr Ann. 2017 Jan 1;46(1):e25-e28. doi: 10.3928/19382359-20161212-01.
Standard first-line therapy for Kawasaki disease (KD) consists of intravenous immunoglobulin (IVIG) and aspirin. Current guidelines recommend 2 g/kg of IVIG and 80 to 100 mg/kg of aspirin administered within the first 10 days of illness. This regimen has marked efficacy in preventing the development of coronary artery aneurysms. Approximately 15% to 20% of treated patients require a second dose of IVIG to control the inflammatory process. The role of adjunctive corticosteroid therapy with IVIG and aspirin is evolving, with Japanese studies showing a clear benefit in those patients at highest risk for development of coronary disease. The challenge in North America has been reliable identification of the highest-risk patients, which still eludes us because the Japanese scoring systems are ineffective in multiethnic populations. Despite its efficacy, the precise mechanism of IVIG's effect in KD is unclear but probably relates to its ability to down-regulate aspects of the up-regulated inflammatory response in patients with KD. [Pediatr Ann. 2017;46(1):e25-e28.].
川崎病（KD）的标准一线治疗包括静脉注射免疫球蛋白（IVIG）和阿司匹林。当前指南推荐在疾病发作的头10天内给予2 g/kg的IVIG和80至100 mg/kg的阿司匹林。该方案在预防冠状动脉瘤形成方面具有显著疗效。约15%至20%的接受治疗的患者需要第二剂IVIG来控制炎症过程。IVIG和阿司匹林联合皮质类固醇治疗的作用正在不断演变，日本的研究表明，这对那些发生冠心病风险最高的患者有明显益处。在北美，面临的挑战是可靠地识别出风险最高的患者，而我们仍然无法做到这一点，因为日本的评分系统在多种族人群中无效。尽管IVIG有效，但其在KD中发挥作用的确切机制尚不清楚，但可能与其下调KD患者上调的炎症反应的某些方面的能力有关。[《儿科年鉴》。2017年；46(1):e25 - e28。]
