Does the SCOPE (Sclerosing Cholangitis Outcomes in PEdiatrics) index effectively predict later liver transplantation in children with sclerosing cholangitis?

Abstract

Background & aims: The SCOPE (Sclerosing Cholangitis Outcomes in Pediatrics) index was developed to provide the first pediatric prognostic score for primary sclerosing cholangitis (PSC), but its efficacy has yet to be confirmed. We aimed to assess its ability to predict liver transplantation (LT) over a 5-year follow-up.

Methods: We retrospectively included PSC-diagnosed patients under 18 years of age from two European tertiary-care centers. The SCOPE index was calculated at diagnosis and at 1, 3, and 5 years post-diagnosis. The ability of the SCOPE index to predict LT was assessed using multivariate Cox regression and ROC curve analysis.

Results: Sixty patients were included. In transplanted patients, the mean SCOPE index at diagnosis was similar to non-transplanted patients, but significantly higher at 1, 3, and 5 years post-diagnosis (p < 0.001, p = 0.009, p = 0.006, respectively). Patients with overlapping autoimmune hepatitis (AIH) had higher SCOPE at diagnosis (p = 0.005), but this difference diminished over time. The SCOPE index was a significant predictor of LT at various time points (HRs: 1.32 to 3.44) and showed good-to-excellent discriminative power (AUC 0.87 at diagnosis; 0.97 at 1 year).

Conclusions: The SCOPE index effectively predicts LT in pediatric PSC, with strong reliability over time. Coexisting AIH may affect score accuracy at diagnosis due to inflammation.

Keywords: Autoimmune hepatitis; Children; Liver transplantation; Primary sclerosing cholangitis.