Abbreviations: AASLD -American Association for the Study of Liver Diseases AIH -autoimmune hepatitis ALT -alanine-aminotransferase ALP -alkaline phosphatase AMA -anti-mitochondrial antibodies ANA -anti-nuclear antibodies AST -aspartate-aminotransferase CI -confidence interval EASL -European Association for the Study of the Liver HCC -hepatocellular carcinoma HLA -human leukocyte antigen IAIHG -International Autoimmune Hepatitis Group INR -international normalised ratio IQR -interquartile range IU/L -international units / liter LKM-1 -liver kidney microsome-1 antibodies MELD -model for end-stage liver disease SD -standard deviation SLE -systemic lupus erythematosus SMA -anti-smooth muscle antibodies UK -United Kingdom Abstract Introduction & AimsSmall studies have found that black patient with autoimmune hepatitis (AIH) patients present with more aggressive disease. We aimed to characterize the presentation and outcome in black and white patients with AIH. MethodsWe performed a retrospective study, collecting information from databases of patients with AIH attending the Institute of Liver studies at King's College Hospital, London (1971-October 2015, the Royal Free Hospital, London (1982 through December 2016) and the multicenter Dutch Autoimmune Hepatitis Study Group cohort (2006-August 2016). We identified 88 black patients with AIH and we compared their clinical characteristics and outcomes to 897 white patients with AIH. ResultsBlack patients presented at a younger age (median 38 years vs 45 years) (P=.007), had higher IgG levels (mean 31.0 mg/dL vs 27.5 mg/dL) (P=.04), but there were no significant differences between groups in auto-antibody profiles, international AIH Group scores, or sex distribution of disease. A higher proportion of black patients had systemic lupus erythematosus (10%) than white patients (2%) (P=<.001). There was no significant difference in proportions of patients with a response to standard therapy (86% for black patients vs 91% for white patients; P=.20) or in rate of relapse (57% vs 50%; P=.3). Despite this, black patients had an increased risk of liver transplantation and liver-related death (hazard ratio 2.4, 95% CI, 1.4-4.0; P<.001). Overall mortality was similar between the two groups. ConclusionIn a comparison of black and white patients with AIH in Europe, we found that black patients present at a younger age, have higher levels of IgG levels, and a greater proportion have SLE. We also found black patients to have a greater risk of liver transplantation and liverrelated mortality, indicating more aggressive disease. 4

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) andThis is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.