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©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
Predictors of survival in autoimmune liver disease overlap syndromes
Dujinthan Jayabalan, Yi Huang, Luis Calzadilla-Bertot, Malik Janjua, Bastiaan de Boer, John Joseph, Wendy Cheng, Simon Hazeldine, Briohny W Smith, Gerry C MacQuillan, Michael C Wallace, George Garas, Leon A Adams, Gary P Jeffrey
Dujinthan Jayabalan, Luis Calzadilla-Bertot, Michael C Wallace, George Garas, Leon A Adams, Gary P Jeffrey, Medical School, University of Western Australia, Nedlands 6009, Western Australia, Australia
Dujinthan Jayabalan, Yi Huang, Luis Calzadilla-Bertot, Malik Janjua, Briohny W Smith, Gerry C MacQuillan, Michael C Wallace, George Garas, Leon A Adams, Gary P Jeffrey, Department of Hepatology, Sir Charles Gairdner Hospital, Nedlands 6009, Western Australia, Australia
Bastiaan de Boer, John Joseph, Department of Anatomical Pathology, PathWest Laboratory Medicine, Nedlands 6009, Western Australia, Australia
Wendy Cheng, Department of Gastroenterology and Hepatology, Royal Perth Hospital, Perth 6000, Western Australia, Australia
Simon Hazeldine, Department of Gastroenterology and Hepatology, Fiona Stanley Hospital, Murdoch 6150, Western Australia, Australia
Gerry C MacQuillan, Medical School, University of Western Australia, Nedlands 6009, Western Australia, Australia
Author contributions: Jayabalan D and Jeffrey GP contributed to study conception and design; Jayabalan D and Huang Y contributed to acquisition of data; Jayabalan D, Huang Y and Calzadilla-Bertot L contributed to analysis and interpretation of results; Jayabalan D contributed to draft manuscript preparation; Jeffrey GP and Adams LA contributed to critical revision of the manuscript for important intellectual content; Jayabalan D, Huang Y, Calzadilla-Bertot L contributed to statistical analysis; Huang Y, Janjua M, de Boer B, Joseph J, Cheng W, Hazeldine S, Smith BW, MacQuillan GC, Wallace MC, Garas G contributed to administrative support; Jeffrey GP contributed to study supervision; All authors reviewed the results and approved the final version of the manuscript.
Institutional review board statement: The study was reviewed and approved by Sir Charles Gairdner Hospital Human Research Ethics Committee (No. RGS0000001775).
Informed consent statement: As the study was retrospective study, the need for informed consent to participate was waived by Sir Charles Gairdner Hospital Human Research Ethics Committee and the Western Australia Department of Health Human Research Ethics Committee.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Data sharing statement: The datasets analysed are available from the corresponding author upon reasonable request at
duji.jayabalan@uwa.edu.au.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
https://creativecommons.org/licenses/by-nc/4.0/ Corresponding author: Dujinthan Jayabalan, BSc, MD, Doctor, Lecturer, Medical School, University of Western Australia, 35 Stirling Highway, Nedlands 6009, Western Australia, Australia.
duji.jayabalan@uwa.edu.au
Received: February 29, 2024
Revised: June 16, 2024
Accepted: June 27, 2024
Published online: September 27, 2024
Processing time: 206 Days and 19 Hours
BACKGROUND
Survival in patients with autoimmune liver disease overlap syndromes (AILDOS) compared to those with single autoimmune liver disease is unclear.
AIM
To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.
METHODS
Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap (AIH-PBC) or autoimmune hepatitis and primary sclerosing cholangitis overlap (AIH-PSC) and were identified from three tertiary centres for this cohort study. Liver-related death or transplantation (liver-related mortality) was determined using a population-based data linkage system. Prognostic scores for liver-related death were compared for accuracy [including liver outcome score (LOS), Hepascore, Mayo Score, model for end-stage liver disease (MELD) score and MELD incorporated with serum sodium (MELD-Na) score].
RESULTS
Twenty-two AILDOS patients were followed for a median of 3.1 years (range, 0.35-7.7). Fourteen were female, the median age was 46.7 years (range, 17.8 to 82.1) and median Hepascore was 1 (range, 0.07-1). At five years post enrolment, 57% of patients remained free from liver-related mortality (74% AIH-PBC, 27% AIH-PSC). There was no significant difference in survival between AIH-PBC and AIH-PSC. LOS was a significant predictor of liver-related mortality (P < 0.05) in patients with AIH-PBC (n = 14) but not AIH-PSC (n = 8). A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients (P = 0.012, log-rank test, 100% sensitivity, 77.8% specificity) (Harrell's C-statistic 0.867). The MELD score, MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.
CONCLUSION
Survival in the rare, AILDOS is unclear. The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients. Further trials investigating predictors of survival in AILDOS are required.
Core Tip: The rare, autoimmune liver disease overlap syndromes (AILDOS), currently have no predictors of survival. AILDOS can be further classified into Autoimmune Hepatitis and Primary Biliary Cholangitis Overlap (AIH-PBC) and Autoimmune Hepatitis and Primary Sclerosing Cholangitis Overlap. Liver-related mortality was defined as liver-related death or liver transplantation. This study validates the liver outcome score as a predictive model of liver-mortality in AIH-PBC patients. The model for end-stage liver disease (MELD) score, MELD incorporated with serum sodium score and Mayo score were not predictive of liver-related mortality in any group.
