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

BACKGROUND

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.

OBJECTIVES

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

DESIGN

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.

METHODS

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.

RESULTS

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).

CONCLUSION

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.