Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2025 Mar;40(3):731-740.
doi: 10.1111/jgh.16850. Epub 2024 Dec 17.

Natural History and Predictors of Clinical Outcomes in Autoimmune Liver Diseases: A Multicenter Study

Dujinthan Jayabalan  1   2 , Yi Huang  2 , Luis Calzadilla-Bertot  1   2 , Leon A Adams  1   2 , Wendy Cheng  3 , Simon Hazeldine  4 , Briohny W Smith  2 , Gerry C MacQuillan  1   2 , Michael C Wallace  1   2 , George Garas  1   2 , Gary P Jeffrey  1   2
Affiliations
Multicenter Study

Natural History and Predictors of Clinical Outcomes in Autoimmune Liver Diseases: A Multicenter Study

Dujinthan Jayabalan et al. J Gastroenterol Hepatol. 2025 Mar.

Abstract

Background and aim: Prognosis in autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) has historically been poor. This multicenter retrospective cohort study investigated the natural history and assessed the predictors of outcomes in patients with AIH, PBC, and PSC.

Methods: AIH, PBC, and PSC patients were identified from the state-wide Hepascore and Clinical Outcome cohort. Overall death or transplant, liver-related mortality (liver-related death or transplant), and liver-related decompensation were determined using a population-based data linkage system. Baseline Liver Outcome Score (LOS), Hepascore, and MELD were examined for predicting outcomes.

Results: Two-hundred thirty-seven AIH patients (24% male, median age 56.6 years [range, 14.3-94.0]), 157 PBC patients (8.3% male, median age 60.5 years [range, 25.6-87.1]), and 167 PSC patients (52.7% male, median age 55.6 years [range, 18.4-88.6]) were enrolled. Five-year transplant-free survival was 88% (95%CI: 81-92%) in AIH, 92% (95%CI: 85-96%) in PBC, and 61% (95%CI: 51-69%) in PSC. PSC had a significantly worse overall death or transplant, liver-related mortality, and liver-related decompensation when compared to AIH and PBC (p < 0.0001). LOS was a significant independent predictor of overall death or transplant, liver-related mortality, and liver-related decompensation among patients with AIH and PBC. LOS was a significant independent predictor of overall death or transplant in patients with PSC, and Hepascore was a significant independent predictor of liver-related mortality and liver-related decompensation.

Conclusions: Outcomes for AIH and PBC are excellent but remain poor in PSC. LOS is a predictor of outcomes in autoimmune liver disease.

Keywords: autoimmune hepatitis; hepascore; primary biliary cholangitis; primary sclerosing cholangitis.

PubMed Disclaimer

References

    1. J. Maggs and S. Cullen, “Management of Autoimmune Liver Disease,” Minerva Gastroenterologica e Dietologica 55, no. 2 (2009): 173–206.
    1. L. Muratori, A. W. Lohse, and M. Lenzi, “Diagnosis and Management of Autoimmune Hepatitis,” BMJ 380 (2023): e070201.
    1. G. R. Locke, 3rd, T. M. Therneau, J. Ludwig, E. R. Dickson, and K. D. Lindor, “Time Course of Histological Progression in Primary Biliary Cirrhosis,” Hepatology 23, no. 1 (1996): 52–56.
    1. T. H. Karlsen, T. Folseraas, D. Thorburn, and M. Vesterhus, “Primary Sclerosing Cholangitis—A Comprehensive Review,” Journal of Hepatology 67, no. 6 (2017): 1298–1323.
    1. L. Bossen, A. Gerussi, V. Lygoura, G. F. Mells, M. Carbone, and P. Invernizzi, “Support of Precision Medicine Through Risk‐Stratification in Autoimmune Liver Diseases—Histology, Scoring Systems, and Non‐Invasive Markers,” Autoimmunity Reviews 17, no. 9 (2018): 854–865.

Publication types

MeSH terms

LinkOut - more resources