Clinical and histological predictors of biliary injury in children with autoimmune liver diseases: development of pediatric biliary indices
DOI:
https://doi.org/10.15574/PS.2025.3(88).4756Keywords:
autoimmune liver diseases, children, biliary phenotype, autoimmune hepatitis, autoimmune sclerosing cholangitis, primary sclerosing cholangitisAbstract
Differentiation between hepatocellular and biliary phenotypes of autoimmune liver diseases (AILD) in children remains challenging due to the lack of validated pediatric scoring systems for biliary injury, which complicates the diagnosis of immune-mediated cholangiopathies.
Aim - to develop pediatric histological and non-invasive biochemical indices to predict the biliary phenotype of AILD in children.
Materials and methods. Children with autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and primary sclerosing cholangitis (PSC) were included. Liver biopsy specimens were evaluated using the Ishak scoring system and the Nakanuma classification. For development of the histological biliary index, 69 children with AILD were analyzed (AIH - n=25; ASC+PSC - n=44). For development of the pediatric non-invasive biliary index, 128 children were included (AIH - n=52; ASC+PSC - n=76). Prediction models were constructed using multivariable logistic regression analysis.
Results. Two multivariable logistic regression models were developed: a non-invasive biochemical model and a histology-based model. The non-invasive pediatric biliary index included sex, alanine aminotransferase (ALT), the gamma-glutamyltransferase to aspartate aminotransferase ratio (GGT/AST), and total cholesterol, and demonstrated good discriminatory performance (AUC=0.84; 95% confidence interval [CI]: 0.77-0.90). The histological biliary index included sex, the GGT/AST ratio, ductopenia, ductular fibrosis, and cholangitis activity, and showed excellent discriminatory performance (AUC=0.95; 95% CI: 0.88-0.99).
Conclusions. The proposed pediatric biliary indices improve diagnostic accuracy for immune-mediated cholangiopathies in children, enable early identification of small bile duct injury, and support more robust stratification of patients according to the biliary phenotype of AILD.
The study was conducted in accordance with the Declaration of Helsinki and approved by the local ethics committee. Written informed consent was obtained from patients or their legal guardians. The authors declare no conflict of interest.
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