川崎病(KD)的最佳疗法为IVIG联合阿司匹林口服,但仍有部分患儿对IVIG治疗无反应,且这部分患儿发生冠状动脉损害的危险性较高,但其发生机制目前仍不明确。患儿年龄较小,IVIG初治时间≤4 d,白细胞计数、中性粒细胞计数、AST及CRP水平升高、血浆清蛋白及血小板减低等是IVIG无反应型KD的危险因素。IVIG无反应型KD的治疗方案目前仍存在争议,再次IVIG治疗是目前多数学者的共识,其他治疗方法如糖皮质激素、英利昔单抗、血浆置换等也在进一步研究中。现从IVIG无反应型KD的发病率、发生机制、危险因素及治疗方面进行综述。 Kawasaki disease (KD) is the best treatment for oral IVIG combined with aspirin, but there are still some children did not respond to IVIG treatment, and this part of children with coronary artery lesions at higher risk, but the mechanism is still not clear . Children younger, IVIG naive time ≤ 4 d, white blood cell count, neutrophil count, AST and CRP levels, plasma albumin and thrombocytopenia are IVIG non-responsive KD risk factors. IVIG non-responsive KD treatment is still controversial, once again IVIG treatment is the consensus of most scholars, other treatments such as glucocorticoids, infliximab, plasma exchange is also under further study. Now from the incidence of IVIG non-responsive KD, its mechanism, risk factors and treatment are reviewed.