Department of Pediatrics, Kaohsiung Veterans General Hospital, Kaohsiung, and National Yang-Ming University School of Medicine, Taipei, Taiwan, ROC.
J Chin Med Assoc. 2011 Nov;74(11):481-4. doi: 10.1016/j.jcma.2011.09.001. Epub 2011 Nov 4.
Kawasaki disease (KD) is acute systemic vasculitis that occurs mainly in infants and children under 5 years of age. The etiology of KD remains unknown. KD is liable to be complicated by coronary artery lesions (CALs), which develop in approximately 15-25% of untreated KD children and in approximately 5% of KD children after intravenous immunoglobulin (IVIG) therapy. A single high dose of IVIG (2 g/kg) is the gold standard therapy in the acute stage of KD. However, approximately 8--38% of children are unresponsive to initial IVIG treatment and at increased risk for CAL development. Anti-inflammatory high doses of aspirin are recommended in conjunction with IVIG, but our study demonstrated that there is no evidence of efficacy in preventing CAL development. The usefulness of steroids in initial therapy for KD or treatment of IVIG-resistant patients is not well established. Other immunosuppressive therapies, including infliximab, have been used in the treatment of refractory KD, but merit additional investigation. Subclinical atherosclerosis may develop early in KD patients, which makes early initiation of therapy to improve chronic inflammation an important issue. Future multicenter studies may help to define the optimal management of KD patients.
川崎病（KD）是一种主要发生在 5 岁以下婴幼儿的急性全身性血管炎。KD 的病因仍不清楚。KD 容易并发冠状动脉病变（CALs），未经治疗的 KD 患儿中约有 15-25%发生 CALs，静脉注射免疫球蛋白（IVIG）治疗后约有 5%发生 CALs。单剂大剂量 IVIG（2g/kg）是 KD 急性期的金标准治疗。然而，约有 8-38%的患儿对初始 IVIG 治疗无反应，发生 CAL 的风险增加。建议在 IVIG 治疗的同时使用抗炎剂量的阿司匹林，但我们的研究表明，其在预防 CAL 发展方面没有疗效证据。皮质类固醇在 KD 的初始治疗或治疗 IVIG 抵抗患者中的作用尚未得到充分证实。其他免疫抑制剂治疗，包括英夫利昔单抗，已用于难治性 KD 的治疗，但值得进一步研究。亚临床动脉粥样硬化可能在 KD 患者中早期发生，因此早期开始治疗以改善慢性炎症是一个重要问题。未来的多中心研究可能有助于确定 KD 患者的最佳治疗方案。
