2018 年第 8 期 第 13 卷

儿童丙种球蛋白无反应型川崎病治疗进展

Progress in the treatment of intravenous immunoglobulin-resistant Kawasaki disease in children

作者：乐园杨曦刘桂英

英文作者：

单位：100029首都医科大学附属北京安贞医院儿科

英文单位：

关键词：川崎病；丙种球蛋白无反应；治疗进展

英文关键词：

• 摘要：

• 川崎病又称皮肤黏膜淋巴结综合征，是一种原因不明的以全身血管炎为主要病变的发热出疹性疾病。病变主要累及全身中、小血管，尤其是冠状动脉损害较多，包括冠状动脉扩张、冠状动脉瘤、冠状动脉狭窄甚至血栓形成。输注丙种球蛋白（IVIG）联合阿司匹林是急性期川崎病的标准治疗方案，但约有10%~20%的川崎病患儿对单次注射IVIG（2 g/kg）联合阿司匹林并无效果，称为IVIG无反应型川崎病。该部分患儿出现冠状动脉损害甚至冠状动脉瘤的风险明显升高，国内外文献报道有多种治疗IVIG无反应型川崎病的方法，包括IVIG追加治疗、糖皮质激素、肿瘤坏死因子α抑制剂（英夫利昔单抗）、阿昔单抗、钙调节神经蛋白抑制剂（环孢素）、降脂药（阿伐他汀）、他丁类药物（乌司他丁）、甲氨蝶呤、抗白细胞介素1（阿那白滞素）、抗CD20单克隆抗体（利妥昔单抗）、血浆置换等。本文就IVIG无反应型川崎病患儿的治疗进展进行综述。

• Kawasaki disease(KD), also known as mucocutaneous lymph node syndrome, is an agnogenic systemic vasculitis manifested as febrile and rush. The disease mainly involves small and medium-sized blood vessels. Coronary artery lesions are common, including coronary artery dilatation, coronary artery aneurysm, coronary artery stenosis and thrombosis. Immunoglobulin(IVIG) infusion combined with aspirin is a standard treatment for acute KD, but single dose of IVIG（2 g/kg） shows no significant effect on 10%-20% children with KD, which is called IVIG-resistant KD. Children with IVIG-resistant KD have a high risk of coronary artery damage especially coronary artery aneurysm. Treatments for IVIG-resistant KD include additional use of IVIG, glucocorticoids, tumor necrosis factor-α inhibitors(infliximab), abciximab, calmodulin inhibitors(cyclosporine), lipid-lowering drugs（alvastatin）, statins(ulinastatin), methotrexate, anti-interleukin-1(anakinra), monoclonal antibody against CD20(rituximab) and plasma exchange. This article reviewed the progress in treatment of IVIG-reactive KD In children.