Cyclosporin A Treatment for Kawasaki Disease Refractory to Initial and Additional Intravenous Immunoglobulin
Cyclosporin A Treatment for Kawasaki Disease Refractory to Initial and Additional Intravenous Immunoglobulin
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DOI：
10.1097/inf.0b013e318220c3cf
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发表时间：
2011-10-01
影响因子：
3.6
通讯作者：
Hata, Akira
中科院分区：
文献类型：
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作者：
Suzuki, Hiroyuki;Terai, Masaru;Hata, Akira
背景：难治性川崎病(KD)目前尚无确切的治疗方法。在这项先导性研究中，我们评价了环孢素A(CyA)治疗难治性KD的临床应用。方法：我们前瞻性地收集了几家医院采用相同方案治疗难治性KD患者的临床资料。难治性KD的定义是在第二次静脉注射免疫球蛋白(2g/kg)结束时持续或复发(腋下温度37.5摄氏度或以上)。结果：2008年1月至2010年6月收治的329例KD患者中，受试者为入选对象。28例难治性KD患者接受环磷酰胺治疗，其中18例(64.3%)在3天内迅速起效，C-反应蛋白下降，另4例在4~5天内转热。但有6例(21.4%)在化疗开始后5天内未退热和/或在5天内退热。虽然9例患者在CyA治疗开始后3~7天出现高钾血症，但未出现心律失常等严重不良反应。4例患者(1.2%)，其中2例在环磷酰胺治疗前和2例在环磷酰胺治疗后发生冠状动脉病变。结论：环磷酰胺治疗被认为是安全和耐受性良好的，是难治性川崎病患者的一种有前途的选择。还需要进一步的研究来明确CyA治疗的最佳剂量、安全性和时机。
Background: 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.Methods: 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 degrees C or more of an axillary temperature) at the end of the second intravenous immunoglobulin (2 g/kg) following the initial one.Results: 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.Conclusion: 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.