Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome:First report of the IAIHG retrospective registry

Dutch AIH Study Group, International Autoimmune Hepatitis Group (2024) Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome:First report of the IAIHG retrospective registry. Hepatology, 79 (3). pp. 538-550. ISSN 0270-9139

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Abstract

Background and Aims: The International Autoimmune Hepatitis Group retrospective registry (IAIHG-RR) is a web-based platform with subjects enrolled with a clinical diagnosis of autoimmune hepatitis (AIH). As prognostic factor studies with enough power are scarce, this study aimed to ascertain data quality and identify prognostic factors in the IAIHG-RR cohort.  Methods: This retrospective, observational, multicenter study included all patients with a clinical diagnosis of AIH from the IAIHG-RR. The quality assessment consisted of external validation of completeness and consistency for 29 predefined variables. Cox regression was used to identify risk factors for liver-related death and liver transplantation (LT).  Results: This analysis included 2559 patients across 7 countries. In 1700 patients, follow-up was available, with a completeness of individual data of 90% (range: 30-100). During a median follow-up period of 10 (range: 0-49) years, there were 229 deaths, of which 116 were liver-related, and 143 patients underwent LT. Non-White ethnicity (HR 4.1 95% CI: 2.3-7.1), cirrhosis (HR 3.5 95% CI: 2.3-5.5), variant syndrome with primary sclerosing cholangitis (PSC) (HR 3.1 95% CI: 1.6-6.2), and lack of complete biochemical response within 6 months (HR 5.7 95% CI: 3.4-9.6) were independent prognostic factors.  Conclusions: The IAIHG-RR represents the world's largest AIH cohort with moderate-to-good data quality and a relevant number of liver-related events. The registry is a suitable platform for patient selection in future studies. Lack of complete biochemical response to treatment, non-White ethnicity, cirrhosis, and PSC-AIH were associated with liver-related death and LT.

Item Type:	Article
Additional Information:	Publisher Copyright: © 2023 American Association for the Study of Liver Diseases.
Uncontrolled Keywords:	hepatology,sdg 3 - good health and well-being ,/dk/atira/pure/subjectarea/asjc/2700/2721
Faculty \ School:	Faculty of Medicine and Health Sciences > Norwich Medical School
Related URLs:	https://www.scopus.com/pages/publication...
Depositing User:	LivePure Connector
Date Deposited:	24 Aug 2026 10:09
Last Modified:	25 Aug 2026 21:10
URI:	https://ueaeprints.uea.ac.uk/id/eprint/104302
DOI:	10.1097/HEP.0000000000000589

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