Department of Pediatrics, Yokohama City University, School of Medicine Yokohama, Japan.
Department of Pediatrics, Yokohama City University Medical Center, Yokohama, Japan.
J Pediatr. 2014 May;164(5):1128-1132.e1. doi: 10.1016/j.jpeds.2014.01.020. Epub 2014 Feb 20.
To evaluate infliximab (IFX) in patients with Kawasaki disease (KD) that was unresponsive to additional intravenous immunoglobulin (IVIG) therapy and subsequent rescue with supplementary plasma exchange (PE) in patients unresponsive to treatment.
We studied 76 patients with KD who received IVIG therapy twice and were unresponsive to additional IVIG.
Seventy were treated with IFX alone (92.1%). Six patients who were unresponsive IFX (7.9%) were further treated by PE. This resulted in disappearance of fever and other clinical symptoms, and improvement of laboratory data. There was no severe life-threatening adverse events.Twelve of the 76 cases had developed coronary artery dilatation, and 3 had coronary artery aneurysm within 1 month of disease onset. At the end of follow-up, in all cases, coronary artery lesions were suppressed or reversed.
Treatment of intractable KD with sequential IVIG, IFX, and PE treatments in a step-wise protocol was effective.
评估英夫利昔单抗（IFX）在对额外静脉注射免疫球蛋白（IVIG）治疗无反应且随后对治疗无反应的患者进行补充血浆置换（PE）进行抢救的川崎病（KD）患者中的作用。
我们研究了 76 名接受过两次 IVIG 治疗且对额外 IVIG 无反应的 KD 患者。
70 名患者单独接受 IFX 治疗（92.1%）。6 名对 IFX 无反应的患者（7.9%）进一步接受了 PE 治疗。这导致发热和其他临床症状消失，实验室数据改善。没有严重的危及生命的不良事件。76 例中有 12 例在发病后 1 个月内发生冠状动脉扩张，3 例发生冠状动脉瘤。在随访结束时，所有病例的冠状动脉病变均得到抑制或逆转。
采用逐步方案进行序贯 IVIG、IFX 和 PE 治疗难治性 KD 是有效的。
