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Lookup NU author(s): Professor David Jones
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© 2023 American Association for the Study of Liver Diseases. 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.
Author(s): Slooter CD, Van Den Brand FF, Lleo A, Colapietro F, Muratori P, Dalekos GN, Lucena MI, Di Zeo-Sanchez DE, Andrade RJ, Montano-Loza AJ, Lytvyak E, Lissenberg-Witte BI, Maisonneuve P, Bouma G, MacEdo G, De Boer YS, Almasio P, Alvarez F, Andrade R, Arikan C, Assis D, Bardou-Jacquet E, Biewenga M, Van Hoek B, De Boer Y, Van Nieuwkerk C, Cancado E, Cazzagon N, Chazouilleres O, Colloredo G, Cuarterolo M, Ines Lopez S, Dalekos G, Gatselis N, Debray D, Robles-Diaz M, Robles-Diaz M, Drenth J, Dyson J, Efe C, Engel B, Ferri S, Lenzi M, Lenzi M, Muratori L, Fontana R, Gerussi A, Invernizzi P, Halilbasic E, Halliday N, Heneghan M, Liberal R, Liberal R, Mieli-Vergani G, Hirschfield G, Horby Jorgensen M, Indolfini G, Iorio R, Jeong S, Jones D, Kelly D, Kerkar N, Kerkar N, Lacaille F, Lammert C, Leggett B, Levy C, Lohse A, De Martin E, McLin V, Milkiewicz P, Mohan N, Nebbia G, Oo Y, Ortega A, Pares A, Pop T, Pratt D, Purnak T, Ranucci G, Rushbrook S, Schramm C, Stattermayer A, Swain M, Tanaka A, Taubert R, Terrabuio D, Terziroli B, Trauner M, Valentino P, Vergani D, Van Den Brand F, Vierling JM, Villamil A, Wahlin S, Ytting H, Zachou K, Zachou K, Zeniya M, Van Gerven N, Van Erpecum K, Den Ouden J, Brouwer J, Vrolijk J, Gevers TJG, Guichelaar M, Bouma G, Schreuder TCMA, Van Der Wouden EJ, Baak LC, Verdonk R, Van Der Meer A, Klemt-Kropp M, Verhagen M, Bhalla A, Kuijvenhoven J
Publication type: Article
Publication status: Published
Journal: Hepatology
Year: 2024
Volume: 79
Issue: 3
Pages: 538-550
Print publication date: 01/03/2024
Acceptance date: 02/04/2018
ISSN (print): 0270-9139
ISSN (electronic): 1527-3350
Publisher: Lippincott Williams and Wilkins
URL: https://doi.org/10.1097/HEP.0000000000000589
DOI: 10.1097/HEP.0000000000000589
PubMed id: 37676683
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