儿童丙种球蛋白无反应型川崎病治疗进展

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(2g/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1VIG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-or 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