Department of Pediatrics, Wakayama Medical University, Wakayama, Japan.
Pediatr Infect Dis J. 2011 Oct;30(10):871-6. doi: 10.1097/INF.0b013e318220c3cf.
There are still no definite treatments for refractory Kawasaki disease (KD). In this pilot study, we evaluated the use of cyclosporin A (CyA) treatment in patients with refractory KD.
We prospectively collected clinical data of CyA treatment (4-8 mg/kg/d, oral administration) for refractory KD patients using the same protocol among several hospitals. Refractory KD is defined as the persistence or recurrence of fever (37.5°C or more of an axillary temperature) at the end of the second intravenous immunoglobulin (2 g/kg) following the initial one.
Subjects were enrolled out of 329 KD patients who were admitted to our 8 hospitals between January 2008 and June 2010. Among a total of 28 patients of refractory KD treated with CyA, 18 (64.3%) responded promptly to be afebrile within 3 days and had decreased C-reactive protein levels, the other 4 became afebrile within 4 to 5 days. However, 6 patients (21.4%) failed to become afebrile within 5 days after the start of CyA and/or high fever returned after becoming afebrile within 5 days. Although hyperkalemia developed in 9 patients at 3 to 7 days after the start of CyA treatment, there were no serious adverse effects such as arrhythmias. Four patients (1.2%), 2 before and the other 2 after the start of CyA treatment, developed coronary arterial lesions.
CyA treatment is considered safe and well tolerated, and a promising option for patients with refractory KD. Further investigations will be needed to clarify optimal dose, safety, and timing of CyA treatment.
目前对于川崎病（KD）的难治性病例仍缺乏确切的治疗方法。在本研究中，我们评估了环孢素 A（CyA）在难治性 KD 患者中的应用。
我们前瞻性地收集了使用相同方案在多家医院治疗难治性 KD 患者的 CyA 治疗（4-8 mg/kg/d，口服）的临床数据。难治性 KD 定义为在初始静脉注射免疫球蛋白（2 g/kg）后第 2 天仍持续或复发发热（腋温 37.5°C 或以上）。
研究对象纳入了 2008 年 1 月至 2010 年 6 月期间在我们 8 家医院就诊的 329 例 KD 患者。在 28 例难治性 KD 患者中，28 例患者接受 CyA 治疗后，18 例（64.3%）在 3 天内迅速退热且 C 反应蛋白水平下降，4 例在 4-5 天内退热。然而，6 例（21.4%）在 CyA 治疗开始后 5 天内未退热且高热在 5 天内退热后再次出现。尽管 9 例患者在 CyA 治疗开始后 3-7 天出现高钾血症，但无心律失常等严重不良反应。4 例患者（1.2%）出现冠状动脉病变，其中 2 例在 CyA 治疗前，2 例在 CyA 治疗后。
CyA 治疗安全且耐受性良好，是难治性 KD 患者的一种有前途的选择。需要进一步研究以明确 CyA 治疗的最佳剂量、安全性和时机。
