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

2021; Wiley; Volume: 9; Issue: 6 Linguagem: Inglês

10.1002/ueg2.12112

2050-6414

Maaike Biewenga, Xavier 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, Ynto 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,

Liver Disease and Transplantation

No prognostic score is currently available for long-term survival in autoimmune hepatitis (AIH) patients.The aim of this study was to develop and validate such a prognostic score for AIH patients at diagnosis.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.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]).A Dutch-Belgian prognostic score for long-term transplant-free survival in AIH patients at diagnosis was developed and validated.