Bellicini Irene, Bainto Emelia, Shimizu Chisato, Burns Jane C, Tremoulet Adriana H
Department of Pediatrics, University of California, San Diego, La Jolla, CA.
Department of Pediatrics, University of California, San Diego, La Jolla, CA; Rady Children's Hospital, San Diego, CA.
J Pediatr. 2025 Apr;279:114479. doi: 10.1016/j.jpeds.2025.114479. Epub 2025 Jan 24.
To describe the clinical course and outcome of 33 patients with Kawasaki disease (KD) treated with cyclosporine (CSA) for coronary artery abnormalities (CAA) or treatment resistance.
Single-center, retrospective study of patients with KD treated from 2013 through 2023 for CAA or treatment resistance. Demographics, laboratory studies, medications, adverse events, and echocardiographic data were analyzed.
Of the 33 KD patients treated with CSA, 25 patients received CSA for CAA and 8 for treatment resistance. Four patients received CSA intravenously initially, followed by oral therapy. Since 2014, all patients received CSA orally only. The target 2-hour postdose level (300-600 ng/ml) was best achieved by dividing 5 mg/kg/day every 12 hours. We developed a standardized treatment protocol based on our experiences with this patient population. The patients treated for CAA all showed improved coronary artery Z-scores at both the 2-week and 6-week follow-up. Two patients experienced significant adverse events. One patient had posterior reversible encephalopathy syndrome, while the other developed Epstein-Barr virus-associated lymphoproliferative disorder. Discontinuation of CSA led to complete recovery in both cases.
CSA was generally well tolerated in patients with KD and CAA or treatment resistance. Our study contributes to the limited literature on CSA use in KD, providing dosing guidance and advocating for cautious monitoring.
描述33例川崎病（KD）患者使用环孢素（CSA）治疗冠状动脉异常（CAA）或治疗抵抗的临床过程及结果。
对2013年至2023年因CAA或治疗抵抗接受治疗的KD患者进行单中心回顾性研究。分析人口统计学、实验室检查、用药情况、不良事件及超声心动图数据。
在33例接受CSA治疗的KD患者中，25例因CAA接受CSA治疗，8例因治疗抵抗接受治疗。4例患者最初静脉注射CSA，随后改为口服治疗。自2014年起，所有患者仅接受口服CSA治疗。通过每12小时给予5mg/kg/天，最能达到目标给药后2小时血药浓度（300 - 600 ng/ml）。我们根据对该患者群体的经验制定了标准化治疗方案。接受CAA治疗的患者在2周和6周随访时冠状动脉Z评分均有所改善。2例患者发生严重不良事件。1例患者出现后部可逆性脑病综合征，另1例发生与爱泼斯坦-巴尔病毒相关的淋巴增殖性疾病。停用CSA后，两例患者均完全康复。
KD合并CAA或治疗抵抗的患者对CSA总体耐受性良好。我们的研究为KD使用CSA的有限文献做出了贡献，提供了给药指导并提倡谨慎监测。
