Development and validation of a prognostic score for long-term transplant-free survival in autoimmune hepatitis type 1

Maaike Biewenga, Xavier P.D.M.J. Verhelst, Martine A.M.C. Baven-Pronk, Hein Putter, Aad P. van den Berg, Karin C.M.J. van Nieuwkerk, Henk R. van Buuren, Gerd Bouma, Ynte S. de Boer, Cedric Simoen, Isabelle Colle, Jeoffrey Schouten, Filip Sermon, Christophe van Steenkiste, Hans van Vlierberghe, Adriaan J. van der Meer, Frederik Nevens, Bart van Hoek*, J. P.H. Drenth, N. M. van GervenU. Beuers, K. J. van Erpecum, J. W. den Ouden, A. Bhalla, J. T. Brouwer, J. M. Vrolijk, G. H. Koek, M. M.J. Guichelaar, E. J. van der Wouden, J. J.M. van Meyel, L. C. Baak, R. C. Verdonk, M. Klemt-Kropp, M. A.M.T. Verhagen, J. Kuijvenhoven, H. M. de Jonge, Dutch Autoimmune Hepatitis Study Group

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Background: No prognostic score is currently available for long-term survival in autoimmune hepatitis (AIH) patients. Objective: The aim of this study was to develop and validate such a prognostic score for AIH patients at diagnosis. Methods: The prognostic score was developed using uni- & multivariate Cox regression in a 4-center Dutch cohort and validated in an independent 6-center Belgian cohort. Results: In the derivation cohort of 396 patients 19 liver transplantations (LTs) and 51 deaths occurred (median follow-up 118 months; interquartile range 60–202 months). In multivariate analysis age (hazard ratio [HR] 1.045; p < 0.001), non-caucasian ethnicity (HR 1.897; p = 0.045), cirrhosis (HR 3.266; p < 0.001) and alanine aminotransferase level (HR 0.725; p = 0.003) were significant independent predictors for mortality or LT (C-statistic 0.827; 95% CI 0.790–0.864). In the validation cohort of 408 patients death or LT occurred in 78 patients during a median follow-up of 74 months (interquartile range: 25–142 months). Predicted 5-year event rate did not differ from observed event rate (high risk group 21.5% vs. 15.7% (95% CI: 6.3%–24.2%); moderate risk group 5.8% versus 4.3% (95% CI: 0.0%–9.1%); low risk group 1.9% versus 5.4% (95% CI: 0.0%–11.4%); C-statistic 0.744 [95% CI 0.644–0.844]). Conclusions: A Dutch-Belgian prognostic score for long-term transplant-free survival in AIH patients at diagnosis was developed and validated.
Original languageEnglish
Pages (from-to)662-671
Number of pages10
JournalUnited European Gastroenterology Journal
Volume9
Issue number6
DOIs
Publication statusPublished - 1 Jul 2021

Keywords

  • AIH
  • autoimmune hepatitis
  • autoimmune liver disease
  • liver transplantation
  • long-term survival
  • mortality
  • prognostic score
  • risk stratification
  • transplant-free survival
  • validation

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