Abstract: Background　Some children with Kawasaki disease（KD） unresponsive to intravenous immunoglobulin（IVIG） also show no response to standard therapy，resulting in a higher risk of developing coronary aneurysm. Biologic drug therapy or high-dose intravenous methylprednisolone pulse therapy（IMPT） is effective，but it is high-cost and induces severe side effects. Thus it is necessary to develop highly effective，yet inexpensive treatments. Objective　To investigate the clinical efficacy and safety of low-dose methylprednisolone combined with IVIG in the treatment of KD unresponsive to IVIG.
Methods　From January 2013 to January 2017，by use of the random number table method，114 cases of KD unresponsive to IVIG were evenly divided into the observation group and control group，receiving a second IVIG with low-dose methylprednisolone，a second IVIG，respectively. Serum C-reactive protein （CRP），interleukin-6 （IL-6），and tumor necrosis factor-alpha（TNF-α） levels before and at 72 hours after this round of treatment were measured for evaluating the treatment response. And the incidence of coronary artery injury before and at 1，3，6，12 months after this round of treatment as well as adverse drug reactions during the treatment were measured for evaluating the treatment safety. Results　The response rate of the observation group was much higher than that of the control group〔91.2% （52/57） vs 75.4% （43/57）〕 （P<0.05）.  Before the treatment，the serum CRP，IL-6，and TNF-α levels were similar in both groups （P>0.05），but they were much lower in the observation group than those of the control group at 72 hours after the treatment （P<0.05）. The incidence of coronary artery injury was similar in both groups before the treatment（P>0.05），but it decreased significantly in the observation group at 1，3 months after treatment（P<0.05），then became similar in both groups at 6，12 months after treatment（P>0.05）． Five cases had hypothermia and one case had sinus bradycardia in the observation group，but they recovered without treatment within 2 to 3 days after onset. Conclusion　For patients with KD unresponsive to IVIG，low-dose methylprednisolone combined with IVIG can significantly improve the clinical efficacy，reduce inflammatory response，and promote the recovery of injured coronary artery with high safety.

Key words: Mucocutaneous lymph node syndrome, Kawasaki disease unresponsive to intravenous immunoglobulin, Methylprednisolone, Intravenous immunoglobulin

摘要： 背景　用标准方案治疗静脉丙种球蛋白（IVIG）无反应型川崎病存在部分患儿治疗无效的问题，此部分患儿形成冠脉瘤的风险增加，给予生物制剂或大剂量甲基泼尼松龙冲击治疗，经济压力及不良反应大，需要制定更为有效的治疗方案。目的　观察小剂量甲基泼尼松龙联合IVIG治疗IVIG无反应型川崎病的疗效和安全性。方法　于2013年1月—2017年1月，采用随机数字表法将114例IVIG无反应型川崎病患儿分为对照组和观察组，每组57例。对照组给予第2次IVIG冲击治疗，观察组在第2次IVIG冲击治疗的同时加用小剂量甲基泼尼松龙治疗。比较两组患儿的治疗有效率，第2次IVIG治疗前及治疗后72 h血清C反应蛋白（CRP）、白介素6（IL-6）、肿瘤坏死因子-α（TNF-α）水平，第2次IVIG治疗前及治疗后1、3、6、12个月时冠状动脉损伤率及药物不良反应。结果　观察组患儿的治疗有效率为91.2%（52/57），高于对照组的75.4%（43/57），差异有统计学意义（P<0.05）。第2次IVIG治疗前两组患儿血清CRP、IL-6、TNF-α水平及冠状动脉损伤率比较，差异无统计学意义（P>0.05）；第2次IVIG治疗后72 h观察组CRP、IL-6、TNF-α水平低于对照组，差异有统计学意义（P<0.05）；第2次IVIG治疗后1、3个月时冠状动脉损伤率观察组低于对照组，差异有统计学意义（P<0.05）；治疗后6、12个月时两组冠状动脉损伤率比较，差异无统计学意义（P>0.05）。观察组5例出现低体温、1例出现窦性心动过缓，2~3 d自行恢复正常。结论　小剂量甲基泼尼松龙联合IVIG治疗IVIG无反应型川崎病，可以明显提高临床疗效，降低炎性反应，促进损伤冠状动脉恢复，且具有较高的安全性。

关键词: 黏膜皮肤淋巴结综合征, 静脉丙种球蛋白无反应型川崎病, 甲基泼尼松龙, 静脉丙种球蛋白