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

• Type: Article in International Scientific Journal
• Date: 2023

Authors

Slooter, CD (Author) Other; van den Brand, FF (Author) Other; Lleo, A (Author) Other; Colapietro, F (Author) Other; Lenzi, M (Author) Other; Muratori, P (Author) Other; Kerkar, N (Author) Other; Dalekos, GN (Author) Other; Zachou, K (Author) Other; Lucena, MI (Author) Other; Robles-Díaz, M (Author) Other; Di Zeo-Sánchez, DE (Author) Other; Andrade, RJ (Author) Other; Montano-Loza, AJ (Author) Other; Lytvyak, E (Author) Other; Lissenberg-Witte, BI (Author) Other; Maisonneuve, P (Author) Other; Bouma, G (Author) Other; Macedo G (Author) FMUP; Liberal, R (Author) Other; de Boer, YS (Author) Other

Journal

Title: Hepatology

Vol. 79

Pages: 538-550

ISSN: 0270-9139

Publisher: Wiley-Blackwell

Indexing

ISI Web of Knowledge - 12 Citations

Scopus - 14 Citations

Other information

Authenticus ID: P-00Z-WDX

DOI: 10.1097/hep.0000000000000589

Abstract (EN): 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.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 13