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Clinical Features of Portal Hypertension and Their Prognostic Implications in Patients With Autoimmune Hepatitis
Lukas Burghart1,2, Sonja Treiber1, David Bauer1,2
1Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria.
Insights
Clinically significant portal hypertension (CSPH) features in autoimmune hepatitis (AIH) patients predict decompensation and death. Regular screening for CSPH in AIH is crucial for timely, effective treatment and improved outcomes.
Area of Science:
- Hepatology
- Internal Medicine
- Immunology
Background:
- Autoimmune hepatitis (AIH) can progress to advanced chronic liver disease (ACLD) with clinically significant portal hypertension (CSPH).
- Understanding the prevalence and prognostic impact of CSPH features in AIH is critical for patient management.
Purpose of the Study:
- To evaluate the prevalence of clinical CSPH features in AIH patients.
- To assess the prognostic impact of CSPH on decompensation, liver transplantation (LTX), and mortality in AIH.
- To identify factors associated with LTX/death and protective elements in AIH patients with CSPH.
Main Methods:
- Retrospective analysis of 271 AIH patients diagnosed with sIAIHG-Score ≥ 6 between 2005 and 2023.
- Multivariable Cox regression analysis to determine the association between CSPH features and adverse outcomes.
- Evaluation of the BAVENO-VII criteria for ruling out CSPH in AIH patients.
Main Results:
- 22.1% of AIH patients had CSPH features at diagnosis, increasing to 38.4% during follow-up.
- Both compensated and decompensated CSPH features significantly increased the risk of LTX/death (aHR: 5.77 and 15.73, respectively).
- The BAVENO-VII criteria identified AIH patients with a low 10-year risk of decompensation (0.8%) and high transplant-free survival (97.8%).
Conclusions:
- Clinical CSPH features in AIH are strong indicators of future hepatic decompensation and mortality risk.
- Regular screening for CSPH in AIH patients is recommended to guide timely and appropriate treatment.
- Early detection and management of CSPH can potentially improve survival and reduce the need for liver transplantation.
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.
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