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

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

Data de publicação: Data Ahead of Print:

Autores da FMUP

  • Manuel Guilherme Gonçalves Macedo

    Autor

Participantes de fora da FMUP

  • Slooter, Charlotte D.
  • van den Brand, Floris F.
  • Lleo, Ana
  • Colapietro, Francesca
  • Lenzi, Marco
  • Muratori, Paolo
  • Kerkar, Nanda
  • Dalekos, George N.
  • Zachou, Kalliopi
  • Lucena, M. Isabel
  • Robles-Diaz, Mercedes
  • Di Zeo-Sanchez, Daniel E.
  • Andrade, Raul J.
  • Montano-Loza, Aldo J.
  • Lytvyak, Ellina
  • Lissenberg-Witte, Birgit I.
  • Maisonneuve, Patrick
  • Bouma, Gerd
  • Liberal, Rodrigo
  • de Boer, Ynto S.
  • Dutch AIH Study Grp
  • Int Autoimmune Hepatitis Grp

Unidades de investigação

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.

Dados da publicação

ISSN/ISSNe:
0270-9139, 1527-3350

Hepatology  John Wiley and Sons Ltd

Tipo:
Article
Páginas:
538-550
PubMed:
37676683
Link para outro recurso:
www.scopus.com

Citações Recebidas na Web of Science: 15

Citações Recebidas na Scopus: 15

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Keywords

  • DIAGNOSIS; SURVIVAL; RACE/ETHNICITY; MANAGEMENT; REMISSION; PROGNOSIS; CIRRHOSIS; CRITERIA; TYPE-1

Campos de Estudo

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