川崎病丙种球蛋白耐药的预测和治疗

The prediction and treatment for intravenous immunoglobulin resistant Kawasaki disease

川崎病是儿童常见的发热性血管炎性疾病,10%~20%的川崎病患儿接受首剂2 g/kg静脉注射丙种球蛋白(IVIG)治疗后失败,这部分患儿称之为静脉注射丙种球蛋白无反应型川崎病(IVIGRKD)。对于IVIGRKD的治疗方案包括第二剂IVIG、第二剂IVIG+糖皮质激素、英夫利昔单抗单次静脉注射、环孢霉素静脉注射或口服、阿那白滞素皮下注射及环磷酰胺静脉注射和血浆置换等。并无证据表明有哪一种治疗方案优于其他治疗方案。因此早期预测及时发现IVIGRKD极为重要。但现有预测模型多数基于回顾性资料统计分析,同时因地域及人种的不同,其应用具有局限性,限制了预测评分系统的进一步推广。

Kawasaki disease(KD)is an acute febrile vasculitis and approximate 10%to 20%children with KD are resistant to initial intravenous immunoglobulin(IVIG)treatment at a dose of 2 g/kg,who are named IVIGRKD.Treatments for IVIGRKD include second dose IVIG,second dose IVIG plus glucocorticoid,single intravenous injection of infliximab,intravenous or oral cyclosporine,subcutaneous injection of Anakinra,intravenous cyclophosphamide,and plasma exchange,etc.No evidence suggests which treatment method is better than the others.Therefore,early prediction and timely identification of IVIGRKD are extremely important.Unfortunately,most of current prediction models are based on the statistical analysis of retrospective data,and due to the discrepancy of region and race,their further application are limited.