# Prognostic value of magnetic resonance imaging in pediatric-onset primary sclerosing cholangitis

## TL;DR

This study shows that repeated MRI-based ANALI scores can predict disease progression in children with primary sclerosing cholangitis, but baseline scores alone are not sufficient.

## Contribution

The study is the first to evaluate the prognostic relevance of ANALI scores in pediatric-onset primary sclerosing cholangitis.

## Key findings

- Baseline ANALI scores alone did not predict adverse outcomes in pediatric-onset PSC.
- Higher follow-up ANALI scores were associated with adverse outcomes like decompensated cirrhosis and liver transplantation.
- Interrater agreement for ANALI scores was excellent for ANALINoGd and substantial for ANALIGd.

## Abstract

The prognostic value of magnetic resonance imaging (MRI) in pediatric-onset primary sclerosing cholangitis (PSC) remains poorly studied. The MRI-based ANALI scores with and without gadolinium (ANALIGd and ANALINoGd) have predicted disease progression and complications in adults, but these scores have not been studied in children.

To assess the prognostic relevance of ANALI scores in pediatric-onset PSC.

This retrospective cohort study was conducted at a single tertiary-care center and included patients with pediatric-onset PSC identified from a national PSC registry.

We included 34 patients with pediatric-onset PSC (median age at diagnosis 14 years, interquartile range (IQR) 12.0–16.3; median follow-up time 10.4 years, IQR 7.0–13.2) who had undergone at least two MRIs. Two expert radiologists evaluated the presence of intrahepatic duct dilatation, hepatic dysmorphia, collateral veins, and parenchymal enhancement heterogeneity, which were used to calculate ANALI scores for the baseline and follow-up MRIs. The longitudinal development of ANALI scores was assessed, and the association between scores and clinical endpoints (decompensated cirrhosis, liver transplantation) was examined using Cox proportional hazards regression. Interrater agreement was evaluated for the total scores and the individual score items.

Disease progression was variable, and baseline ANALI scores alone did not predict adverse outcomes. When follow-up scores were also assessed, higher scores were associated with outcomes (hazard ratio (HR) per 1-point increase in ANALINoGd 1.59, 95% confidence interval (CI) 1.15–2.20; p = 0.005; HR per 1-point increase in ANALIGd 3.03, 95% CI 1.06–8.70, p = 0.039). Interrater agreement was excellent for ANALINoGd (0.89, 95% CI 0.83–0.95) and substantial for ANALIGd (0.73, 95% CI 0.62–0.84).

ANALI scores were associated with adverse outcomes in pediatric-onset PSC when assessed repeatedly during follow-up; baseline measurements alone may have limited prognostic value in the pediatric population.

Prognostic value of magnetic resonance imaging in pediatric-onset primary sclerosing cholangitis

This study evaluated the performance of magnetic resonance imaging (MRI) in predicting disease progression and complications in children with a rare liver disease, primary sclerosing cholangitis (PSC). In adult patients with PSC, MRI-based ANALI scores assessed at the time of diagnosis have associated with disease progression and complications, including cirrhosis decompensation and the need of liver transplantation. We examined the performance of ANALI scores in 34 patients with pediatric-onset PSC who had undergone at least two MRIs. Two radiologists examined specific liver features on the scans, and ANALI scores were calculated for the baseline and follow-up MRIs. Findings showed that baseline ANALI scores alone did not predict complications, but when follow-up scores were also assessed, higher scores were associated with outcomes. Our results suggest that ANALI scores are useful for risk stratification in patients with pediatric-onset PSC when assessed repeatedly during follow-up.