Longitudinal Comparison of Currently Used Risk Scores for Prognostication of Primary Sclerosing Cholangitis (PSC) in a Hungarian Bicenter PSC Cohort

Vén, Péter László and Tornai, Dávid and Tóth, Bence and Vitális, Zsuzsanna and Tornai, István and Tornai, Tamás and Pár, Gabriella and Papp, Mária (2025) Longitudinal Comparison of Currently Used Risk Scores for Prognostication of Primary Sclerosing Cholangitis (PSC) in a Hungarian Bicenter PSC Cohort. DIAGNOSTICS, 15 (17). ISSN 2075-4418

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Abstract

Background/Objectives: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with limited epidemiological data from Central–Eastern Europe. This study characterized a Hungarian PSC cohort, comparing patients with and without inflammatory bowel disease (IBD), and longitudinally evaluated the predictive efficacy of established prognostic scores (Mayo Risk Score, Amsterdam-Oxford Model [AOM], UK-PSC short/long). Methods: Data from 135 PSC patients (median diagnosis age 31 years, 57.7% male) were collected yearly at two Hungarian centers, with a median follow-up of 8.8 years. Outcomes included liver transplantation (LT) and liver-related death. Prognostic value of baseline clinical scores was assessed for 2-, 5-, 8-, and 10-year composite outcome. Results: PSC-IBD patients (54.1%) were younger with higher baseline Mayo and AOM scores, and had increased rates of colorectal carcinoma (8.22% vs. 0.00%) and liver transplantation (26.03% vs. 9.68%) within 10 years than PSC-only patients. There were no differences in liver-related mortality or composite outcomes between the groups. All prognostic scores showed good short-term predictive ability for poor outcomes (AUROC at 2 years: 0.858–0.958), which diminished over time (AUROC at 10 years: 0.708–0.756). The AOM demonstrated the most consistent performance. Persistent alkaline phosphatase (ALP) elevation (≥2.2×ULN) 2 years post-diagnosis, despite ursodeoxycholic acid therapy, strongly predicted 10-year adverse outcomes (HR: 3.927, p < 0.001), outperforming formal scoring systems (HR: 2.688–1.522). Conclusions: While PSC-IBD patients had more CRC and liver transplantation, overall transplantation-free survival was similar to PSC-only patients. Prognostic utility of current scores declines with longer follow-up; AOM was most stable. Sustained ALP elevation is a robust long-term prognostic indicator.

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