Abstract: Objective To investigate the prevalence rate and clinical significance of plasma antinuclear antibody (ANA) and antineutrophil cytoplasmic antibody (ANCA) in patients with biliary atresia (BA). Methods The clinical and follow-up data of BA patients underwent liver transplantation (LT) from April 2017 to October 2020 were retrospectively collected. The plasma ANA were semi-quantitatively measured and ANCA were qualitatively detected, and the correlation with laboratory parameters, severity, pathological histological features and post-LT complications were analyzed. Rank sum test was used for continuous data and Chi-square test was used for categorical data. Correlations were evaluated by the Spearman's correlation analysis. Results The prevalence rate of ANA was 3.3% (3/90), and the ANA ratio decreased with the length of native liver survival (R=-0.326). There was a positive correlation between the ANA ratio and Child-Pugh score (R = 0.230), as well as score of pediatric model of end-stage liver disease (R=0.257). The ANA ratio was significantly higher in patients with cirrhosis (0.24 vs 0.20, P=0.016) and the ratio increased with higher degree of ductular cholestasis (P=0.040). In addition, the ANA ratio was significantly higher in patients with CMV infection post-LT (0.31 vs 0.22, P=0.034). The ANCA positive rate was 10.2% (10/98). Prior-LT, plasma Albumin (Alb) and cholinesterase (CHE) level in ANCA-positive patients was significantly lower than that in ANCA-negative patients (Alb: 30.4 vs 34.3 g/L, P=0.018; CHE: 2.6 vs 3.9 KU/L, P=0.009). Conclusion The prevalence rate of ANCA and ANA of patients with BA is low, but it correlates with liver synthetic function, fibrosis stage and post-LT CMV infection. Autoantibodies may be of clinical value for the assessment of BA severity and post-LT prognosis.

Key words: Biliary atresia (BA), Antinuclear antibody (ANA), Antineutrophil cytoplasmic antibody (ANCA), Prognosis, Liver transplantation (LT)