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

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