川畸病(KD)是一种病因不明,以全身血管炎为主要病变的急性发热出疹性疾病。主要累及中小动脉,特别是冠状动脉。静脉注射丙种球蛋白(IVIG)无反应型KD发病机制亦不清楚,因此,治疗难度较大。现就目前IVIG无反应型KD的治疗,包括:再次IVIG治疗、糖皮质激素(GCS)、血浆置换(PE)、乌司他汀及英昔单抗等的新认识作一综述。 Kawasaki disease (KD) is an acute etiologic and rash disease characterized by an unknown etiology and systemic vasculitis. Mainly involving small and medium arteries, especially the coronary arteries. Intravenous gamma globulin (IVIG) non-reactive KD pathogenesis is not clear, therefore, the treatment is more difficult. The current treatment of IVIG nonresponsive KDs is reviewed, including: new insights into IVIG treatment, glucocorticoid (GCS), plasma exchange (PE), ulinastatin, and infliximab.