Abstract: Objective　To investigate the relationship between microtubule-associated protein l light chain 3B (LC3-II) and the inflammatory response of Kawasaki disease (KD). Methods　Thirty-nine cases of acute KD before intravenous administration of immunoglobulin were enrolled. According to the results of echocardiography, the 39 cases were furtherly divided into KD with coronary artery lesion (CAL, 20 cases) group and KD with non-CAL (NCAL, 19 cases) group. At the same time, 12 healthy children were selected as controls. Serum samples were collected and cultured in vitro by human coronary artery endothelial cells (HCAEC) for 12 h. The LC3-II protein and mRNA expression of HCAEC were detected by Western-blotting and Q-PCR respectively. Results　The LC3-II protein and mRNA expression in CAL group and NCAL group were significantly higher than those in control group, the LC3-II protein and mRNA expression in CAL group was higher than those in NCAL group, and both differences were statistically significant (P<0.05). Conclusions　Autophagy may be involved in the inflammatory response of KD in acute phase, which may be related to endothelial cell lesions of coronary artery in children with KD.