Lack of complete biochemical response in autoimmune hepatitis leads to adverse outcome: first report of the IAIHG retrospective registry.
Journal
Hepatology (Baltimore, Md.)
ISSN: 1527-3350
Titre abrégé: Hepatology
Pays: United States
ID NLM: 8302946
Informations de publication
Date de publication:
04 Sep 2023
04 Sep 2023
Historique:
received:
05
02
2023
accepted:
02
08
2023
medline:
7
9
2023
pubmed:
7
9
2023
entrez:
7
9
2023
Statut:
aheadofprint
Résumé
The International Autoimmune hepatitis (AIH) Group retrospective registry (IAIHG-RR) is a web-based platform with subjects enrolled with a clinical diagnosis of 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. This retrospective, observational, multicenter study included all patients with a clinical diagnosis of AIH from the IAIHG-RR. Quality assessment consisted of external validation of completeness and consistency for 29 pre-defined variables. Cox regression was used to identify risk factors for liver-related death and liver transplantation (LT). 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. 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 liver transplantation.
Sections du résumé
BACKGROUND AND AIM
OBJECTIVE
The International Autoimmune hepatitis (AIH) Group retrospective registry (IAIHG-RR) is a web-based platform with subjects enrolled with a clinical diagnosis of 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
METHODS
This retrospective, observational, multicenter study included all patients with a clinical diagnosis of AIH from the IAIHG-RR. Quality assessment consisted of external validation of completeness and consistency for 29 pre-defined variables. Cox regression was used to identify risk factors for liver-related death and liver transplantation (LT).
RESULTS
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.
CONCLUSION
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 liver transplantation.
Identifiants
pubmed: 37676683
doi: 10.1097/HEP.0000000000000589
pii: 01515467-990000000-00557
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Investigateurs
N van Gerven
(N)
K van Erpecum
(K)
J den Ouden
(J)
J Brouwer
(J)
J Vrolijk
(J)
T Gevers
(T)
J Drenth
(J)
M Guichelaar
(M)
G Bouma
(G)
T C M A Schreuder
(TCMA)
E J van der Wouden
(EJ)
L C Baak
(LC)
R Verdonk
(R)
A van der Meer
(A)
M Klemt-Kropp
(M)
M Verhagen
(M)
A Bhalla
(A)
J Kuijvenhoven
(J)
Informations de copyright
Copyright © 2023 American Association for the Study of Liver Diseases.