TY - JOUR
T1 - Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome
T2 - First report of the IAIHG retrospective registry
AU - Slooter, Charlotte D.
AU - van den Brand, Floris F.
AU - Lleo, Ana
AU - Colapietro, Francesca
AU - Lenzi, Marco
AU - Muratori, Paolo
AU - Kerkar, Nanda
AU - Dalekos, George N.
AU - Zachou, Kalliopi
AU - Lucena, M. Isabel
AU - Robles-Díaz, Mercedes
AU - di Zeo-Sánchez, Daniel E.
AU - Andrade, Raúl J.
AU - Montano-Loza, Aldo J.
AU - Lytvyak, Ellina
AU - Dutch AIH Study Group
AU - Lissenberg-Witte, Birgit I.
AU - International Autoimmune Hepatitis Group
AU - Maisonneuve, Patrick
AU - Bouma, Gerd
AU - Dutch AIH Study Group and International Autoimmune Hepatitis Group
AU - Macedo, Guilherme
AU - Liberal, Rodrigo
AU - de Boer, Ynto S.
N1 - Publisher Copyright: Copyright © 2023 American Association for the Study of Liver Diseases.
PY - 2024/3/1
Y1 - 2024/3/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85185343173&partnerID=8YFLogxK
U2 - 10.1097/HEP.0000000000000589
DO - 10.1097/HEP.0000000000000589
M3 - Article
C2 - 37676683
SN - 0270-9139
VL - 79
SP - 538
EP - 550
JO - Hepatology (Baltimore, Md.)
JF - Hepatology (Baltimore, Md.)
IS - 3
ER -