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Observational Study
. 2024 Mar 1;79(3):538-550.
doi: 10.1097/HEP.0000000000000589. Epub 2023 Sep 4.

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

Charlotte D Slooter  1 , Floris F van den Brand  1 , Ana Lleo  2   3 , Francesca Colapietro  2   3 , Marco Lenzi  4 , Paolo Muratori  5 , Nanda Kerkar  6 , George N Dalekos  7 , Kalliopi Zachou  7 , M Isabel Lucena  8 , Mercedes Robles-Díaz  8 , Daniel E Di Zeo-Sánchez  8 , Raúl J Andrade  8 , Aldo J Montano-Loza  9 , Ellina Lytvyak  9 , Birgit I Lissenberg-Witte  10 , Patrick Maisonneuve  11 , Gerd Bouma  1 , Guilherme Macedo  12 , Rodrigo Liberal  12 , Ynto S de Boer  1 ; Dutch AIH Study Group; International Autoimmune Hepatitis Group; Dutch AIH Study Group and International Autoimmune Hepatitis Group
Collaborators, Affiliations
Observational Study

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

Charlotte D Slooter et al. Hepatology. .

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.

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References

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