Background: Concurrent extrahepatic autoimmune disease (CEHAID) associated with

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Introduction & Aims Small 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. Methods We performed a retrospective study, collecting information from databases of patients with

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 & Aims: High IgG levels are considered a hallmark of autoimmune hepatitis (AIH). A subgroup of patients with AIH has IgG within the normal range despite evidence of clinical disease activity. The clinical significance of this biomarker has not been explored. Methods: In a European multicentre study we compared biochemical, clinical and histological features from patients with AIH and normal IgG-values at diagnosis to an age-and sex-matched control group of patients with typical AIH presenting with elevated IgG. Data were assessed at diagnosis, after 12 months of therapy and at last follow-up. Results: Out of 1,318 patients with AIH, 130 (10%) had normal IgG at presentation. Histological and biochemical parameters at diagnosis, as well as treatment response, showed no difference between groups. Stable remission off treatment was achieved more commonly in the normal IgG group than in the typical AIH group (24 vs. 8%; p = 0.0012). Patients of the control group not only had higher IgG levels (29.5 ± 5.8 vs. 12.5 ± 3.2 g/L; p <0.0001), but also a higher IgG/IgA ratio (9.3 ± 6.9 vs. 5.4 ± 2.4; p <0.0001) at diagnosis. The IgG/IgA ratio only declined in patients with typical AIH and was no longer different between groups after 12 months (6.3 ± 4.3 vs. 5.5 ± 2.2; p = 0.1), indicating a selective increase of IgG in typical AIH and its suppression by immunosuppression. Autoantibody titres were higher in the typical AIH group, but not when controlled for IgG levels. Conclusions: Compared to AIH with typical biochemical features, patients with normal IgG levels at diagnosis (i) show similar biochemical, serological and histological features and comparable treatment response, (ii) appear to lack the selective elevation of serum IgG levels observed in typical active AIH disease, (iii) may represent a subgroup with a higher chance of successful drug withdrawal.