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. 2026 Jan;63(2):264-275.
doi: 10.1111/apt.70349. Epub 2025 Aug 28.

Clinical Features of Portal Hypertension and Their Prognostic Implications in Patients With Autoimmune Hepatitis

Lukas Burghart  1   2 , Sonja Treiber  1 , David Bauer  1   2 , Emina Halilbasic  1   3 , Benedikt S Hofer  1   4   5 , Mattias Mandorfer  1   3   4 , Michael Gschwantler  2   6 , Caroline Schwarz  1   2 , Michael Trauner  1   3 , Thomas Reiberger  1   3   4   5 , Albert F Stättermayer  1   3
Affiliations

Clinical Features of Portal Hypertension and Their Prognostic Implications in Patients With Autoimmune Hepatitis

Lukas Burghart et al. Aliment Pharmacol Ther. 2026 Jan.

Abstract

Background and aims: Autoimmune hepatitis (AIH) may progress to advanced chronic liver disease (ACLD) with clinically significant portal hypertension (CSPH). In this study, we evaluated the prevalence of different clinical CSPH features and their prognostic impact regarding decompensation, liver transplantation (LTX) and death in patients with AIH.

Method: Patients with confirmed AIH diagnosis (sIAIHG-Score ≥ 6) managed at the Vienna General Hospital between 2005 and 2023 were retrospectively analysed.

Results: Among 271 included patients (76.4% female) with AIH, n = 60 (22.1%) presented clinical features of CSPH at diagnosis. During a median follow-up of 7.2 (IQR 2.9-12.7) years, the proportion with CSPH features increased to n = 104 (38.4%). In a multivariable cox regression analysis, both compensated (aHR: 5.77, 95% CI: [1.47-22.71], p = 0.012) and decompensated features of CSPH (aHR: 15.73, 95% CI: [4.17-59.33], p < 0.0001) were associated with an increased risk of LTX/death, whereas complete biochemical response and higher albumin levels were identified as protective factors. The BAVENO-VII criteria for ruling-out CSPH (liver stiffness < 15 kPa and platelet count ≥ 150 G/L) identified AIH patients with a negligible 10Y cumulative incidence of hepatic decompensation (0.8%) and a favourable 10Y transplant-free survival (97.8%). Overall, n = 16 (5.9%) patients died, with n = 10 deaths caused by CSPH-related complications.

Conclusion: In patients with AIH, clinical features of CSPH reflect the risk of future hepatic decompensation and mortality. Hence, regular screening for CSPH in AIH patients seems warranted to ensure timely initiation of adequate CSPH-directed treatment.

Keywords: ascites; autoimmune hepatitis; liver stiffness; platelet count; portal hypertension; splenomegaly; varices.

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Conflict of interest statement

Dr. Burghart received grant support from Gilead and travel support from Ipsen, Lilly and Gilead. Dr. Treiber has nothing to declare. Dr. Bauer received travel support from Gilead and AbbVie; speaking honoraria from AbbVie and Siemens and grant support from Gilead and AbbVie. Dr. Halilbasic received travel support from Ipsen. Dr. Hofer received travel support from Ipsen. Prof Mandorfer served as a speaker and/or consultant and/or advisory board member for AbbVie, Gilead, Collective Acumen, and W. L. Gore & Associates, Takeda and received travel support from AbbVie, Bristol‐Myers Squibb and Gilead. Prof Gschwantler received grant support from AbbVie, Gilead and MSD; speaking honoraria from AbbVie, Gilead, Janssen, Roche, Intercept and MSD; consulting/advisory board fees from AbbVie, Gilead, Janssen, Roche, Intercept, Norgine, AstraZeneca, Falk, Shionogi and MSD; and travel support from AbbVie and Gilead. DDr Schwarz received travel support from Gilead, AbbVie, Galápagos and Gebro; speaking honoraria from AbbVie and Gilead; and payments for consulting from Gilead. Prof Trauner received grant support from Albireo, Alnylam, Cymabay, Falk, Gilead, Genentech, Intercept, MSD, Takeda and UltraGenyx; honoraria for consulting from Abbvie, Albireo, Boehringer‐Ingelheim, BiomX, Falk, Genfit, Gilead, Hightide, Intercept, Ipsen, Jannsen, Mirum, MSD, Novartis, Phenex, Pliant, Regulus, Siemens and Shire; speaker fees from Albireo, Bristol‐Myers Squibb, Falk, Gilead, Intercept, Ipsen, MSD and Madrigal, as well as travel support from AbbVie, Falk, Gilead and Intercept. He is also co‐inventor on patents on the medical use of norUDCA/norucholic acid filed by the Medical University of Vienna. Prof Reiberger received grant support from Abbvie, Boehringer‐Ingelheim, Gilead, MSD, Philips Healthcare, Gore; speaking honoraria from Abbvie, Gilead, Gore, Intercept, Roche, MSD; consulting/advisory board fee from Abbvie, Bayer, Boehringer‐Ingelheim, Gilead, Intercept, MSD, Siemens; and travel support from Boehringer‐Ingelheim, Gilead and Roche. Dr. Stättermayer consults for, advises, and/or is on the speakers' bureau for Boehringer‐Ingelheim, Gilead, and MSD.

Figures

FIGURE 1
FIGURE 1
Patient characteristics at baseline. ACLD and CSPH status at baseline in 271 patients with autoimmune hepatitis presenting at the Vienna General Hospital between Q1‐2005 and Q4‐2023.
FIGURE 2
FIGURE 2
Liver disease state and CSPH features in AIH. (A) Absolute N of patients presenting without advanced chronic liver disease (non‐ACLD) vs. with compensated ACLD (cACLD) but without features of clinically significant portal hypertension (CSPH) vs. with cACLD and CSPH features vs. with decompensated ACLD (dACLD). (B) Absolute N of CSPH features observed during the study period. (C) Forrest‐plot with results from three different, time dependent, multivariable cox proportional hazards models (separate model for each CSPH feature to avoid collinearity) in which the HR of each presented CSPH feature was adjusted for MELD‐3.0 (used because MELD‐3.0 additionally includes sex and albumin) and biochemical response.
FIGURE 3
FIGURE 3
Simon–Makuch plots with corresponding number at risk tables, illustrating cumulative incidence of decompensation (upper) as well as transplant‐free survival (lower) across different ACLD stages in patients with AIH while accounting for the circumstance that patients may transition between different ACLD stages, thus giving a more accurate estimate of cumulative incidence of decompensation as well as transplant‐free survival.
FIGURE 4
FIGURE 4
Transplant‐free survival according to ACLD status and CSPH features. (A) Bar chart illustrating the relative frequency of liver transplantation or death (whichever came first) in patients with and without a respective CSPH feature. (B) Forrest‐plot with results from 6 different multivariable cox proportional hazards models (separate model for each CSPH feature to avoid collinearity) in which the HR of each presented CSPH feature was adjusted for MELD‐3.0 (used because MELD‐3.0 additionally includes sex and albumin), age and biochemical response. Data are presented as adjusted HR with 95% CI. (C) Forrest‐plot of a multivariate cox proportional hazards model including MELD‐3.0, age in years, biochemical response, compensated ACLD with CSPH and dACLD (as defined above). Data are presented as adjusted HR with 95% CI.
FIGURE 5
FIGURE 5
BAVENO‐VII CSPH rule‐out criteria. (A, B) Development of liver stiffness thresholds, from baseline to 6 months after AIH therapy initiation with the corresponding course in alanine aminotransferase thresholds. (C) Cumulative incidence of decompensation in patients fulfilling the BAVENO‐VII CSPH rule‐out criteria at month 6 after AIH diagnosis (LSM < 15 kPa and PLT ≥ 150 G/L) vs. those who do not fulfil these criteria. Log‐rank test was used to test for statistical significance. (D) Liver transplant‐free survival in patients fulfilling the BAVENO‐VII CSPH rule‐out criteria at month 6 after AIH diagnosis (LSM < 15 kPa and PLT ≥ 150 G/L) vs. those who do not fulfil these criteria. Log‐rank test was used to test for statistical significance.
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References

    1. Ngu J. H., Bechly K., Chapman B. A., et al., “Population‐Based Epidemiology Study of Autoimmune Hepatitis: A Disease of Older Women?,” Journal of Gastroenterology and Hepatology 25, no. 10 (2010): 1681–1686, 10.1111/j.1440-1746.2010.06384.x. - DOI - PubMed
    1. Mack C. L., Adams D., Assis D. N., et al., “Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases,” Hepatology 72, no. 2 (2020): 671–722, 10.1002/hep.31065. - DOI - PubMed
    1. European Association for the Study of the Liver , “EASL Clinical Practice Guidelines for the Management of Patients With Decompensated Cirrhosis,” Journal of Hepatology 69, no. 2 (2018): 406–460, 10.1016/j.jhep.2018.03.024. - DOI - PubMed
    1. Königshofer P., Hofer B. S., Brusilovskaya K., et al., “Distinct Structural and Dynamic Components of Portal Hypertension in Different Animal Models and Human Liver Disease Etiologies,” Hepatology 75, no. 3 (2021): 610–622, 10.1002/hep.32220. - DOI - PMC - PubMed
    1. Villanueva C., Albillos A., Genescà J., et al., “β Blockers to Prevent Decompensation of Cirrhosis in Patients With Clinically Significant Portal Hypertension (PREDESCI): A Randomised, Double‐Blind, Placebo‐Controlled, Multicentre Trial,” Lancet 393, no. 10181 (2019): 1597–1608, 10.1016/S0140-6736(18)31875-0. - DOI - PubMed

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