Journal Article

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

Nieuwkerk, Karin C.M.J., Baven‐Pronk, Martine A.M.C., Putter, Hein, Buuren, Henk R., Sermon, Filip, Meer, Adriaan J., Steenkiste, Christophe, Berg, Aad P., Nevens, Frederik, Bouma, Gerd, Colle, Isabelle, Schouten, Jeoffrey, Boer, Ynte S., Vlierberghe, Hans, Biewenga, Maaike, Verhelst, Xavier P.D.M.J., Simoen, Cedric, Hoek, Bart

2021

Overview

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.

• Publisher: John Wiley & Sons, Inc,John Wiley and Sons Inc
• Subject: Adolescent / Adult / Age / Aged / Aged, 80 and over / AIH / autoimmune hepatitis / autoimmune liver disease / Belgium - epidemiology / Biopsy / Child / Child, Preschool / Clinical trials / Cohort Studies / Decision Support Techniques / Disease Progression / Disease-Free Survival / Ethnicity / Female / Hepatitis / Hepatitis, Autoimmune - complications / Hepatitis, Autoimmune - mortality / Hepatitis, Autoimmune - therapy / Hepatobiliary / Humans / Liver cancer / Liver cirrhosis / Liver Cirrhosis - etiology / Liver Cirrhosis - mortality / liver transplantation / Liver Transplantation - statistics & numerical data / Liver transplants / long‐term survival / Male / Medical prognosis / Middle Aged / Mortality / Multivariate Analysis / Netherlands - epidemiology / Original / Outpatient care facilities / Patients / Prognosis / prognostic score / Proportional Hazards Models / Remission (Medicine) / Reproducibility of Results / Risk Assessment / Risk Factors / risk stratification / Statistical analysis / Steroids / Survival analysis / Time Factors / transplant‐free survival / validation / Variables / White people / Young Adult