初始剂量丙种球蛋白无反应性川崎病的治疗进展

The Treatment of Kawasaki Disease Unresponsive to Initial Dose of Intravenous Immunoglobulin

虽然静脉用丙种球蛋白(IVIG)可以有效的治疗川崎病(KD),但仍有10%~20%的KD患儿对初始剂量IVIG治疗无反应,使他们合并冠状动脉病变(CAL)的风险增加。对初始剂量丙种球蛋白无反应性川崎病的治疗方案仍有争议。其他疗法主要包括再次输注IVIG、糖皮质激素药物、TNF-α抑制剂、钙调磷酸酶抑制剂、他汀类药物、MTX治疗、血浆置换等。本文综述初始剂量IVIG无反应性KD的治疗进展。

Although intravenous immunoglobulin（IVIG） is highly effective in Kawasaki disease（KD）,mechanisms are not understood and 10-20% of patients are treatment-resistant,manifesting a higher rate of coronary artery aneurysms.The optimal treatment for IVIG-nonresponsive KD remains controversial.Management options include further dose（s） of IVIG,corticosteroids,TNF-α blockade,Calcineurin inhibitors,statins,MTX tierapy,plasmapheresis.This anicle reviewed the progress in the treatment of KD unresponsive to initial dose of IVIG.