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

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.