Department of Pediatrics, Kitasato University School of Medicine, 1-15-1 Kitasato Mimami-ku, Sagamihara-shi, Kanagawa-ken 252-0374, Japan.
Pediatrics. 2012 Jan;129(1):e17-23. doi: 10.1542/peds.2011-0148. Epub 2011 Dec 5.
This study examined the clinical efficacy and safety of intravenous methylprednisolone-pulse plus intravenous immunoglobulin (IVIG) combination therapy (IVMP+IVIG) for the initial treatment of patients predicted to have refractory Kawasaki disease (KD).
One hundred twenty-two patients with KD were studied at Kitasato University. Refractory KD was predicted at diagnosis using the Egami score, and the patients were randomly divided to receive either IVMP+IVIG or IVIG alone. The Egami score is used to predict refractory KD patients before treatment using the patient's age, days of illness, platelet count, C-reactive protein, and alanine aminotransferase level (cutoff: ≥3 points; 78% sensitivity and 76% specificity).
Forty-eight patients (39.3%) were predicted to have refractory KD on the basis of the Egami score. The predicted IVIG responders (n = 74) received the standard therapy. The 48 predicted refractory KD patients were randomly assigned to a single-IVIG group (n = 26) or an IVMP+IVIG group (n = 22). Nineteen of the 22 patients (86.4%) in the IVMP+IVIG group had a prompt defervescence compared with 6 of the 26 patients (23.1%) in the single-IVIG group. The number of patients who had a z score ≥2.5 at 1 month was significantly higher in the single-IVIG group than in the IVMP+IVIG group. No serious adverse events were observed in either treatment group.
This study demonstrated that IVMP+IVIG therapy is safe and effective for KD patients predicted as refractory.
本研究旨在探讨静脉注射甲基泼尼松龙脉冲联合静脉注射免疫球蛋白（IVMP+IVIG）治疗方案对预测为难治性川崎病（KD）的初始治疗的临床疗效和安全性。
在桂由美大学，研究了 122 例 KD 患者。在治疗前使用 Egami 评分预测难治性 KD，患者随机分为 IVMP+IVIG 组或 IVIG 组。Egami 评分用于预测治疗前患者的难治性 KD，评分基于患者年龄、发病天数、血小板计数、C 反应蛋白和丙氨酸氨基转移酶水平（临界值：≥3 分；敏感性 78%，特异性 76%）。
根据 Egami 评分，48 例（39.3%）患者预测为难治性 KD。预测 IVIG 反应者（n=74）接受标准治疗。48 例预测难治性 KD 患者随机分为单 IVIG 组（n=26）或 IVMP+IVIG 组（n=22）。IVMP+IVIG 组 22 例患者中有 19 例（86.4%）迅速退热，而单 IVIG 组 26 例患者中有 6 例（23.1%）。单 IVIG 组患者在 1 个月时有 z 评分≥2.5 的患者数量明显高于 IVMP+IVIG 组。两组治疗均未发生严重不良事件。
本研究表明，IVMP+IVIG 治疗对预测为难治性的 KD 患者是安全有效的。
