Background and Aims: Autoimmune hepatitis (AIH) is a rare chronic liver disease of unknown aetiology; the risk of hepatocellular carcinoma (HCC) remains unclear and risk factors are not well... Show moreBackground and Aims: Autoimmune hepatitis (AIH) is a rare chronic liver disease of unknown aetiology; the risk of hepatocellular carcinoma (HCC) remains unclear and risk factors are not well-defined. We aimed to investigate the risk of HCC across a multicentre AIH cohort and to identify predictive factors. Methods: We performed a retrospective, observational, multicentric study of patients included in the International Autoimmune Hepatitis Group Retrospective Registry. The assessed clinical outcomes were HCC development, liver transplantation, and death. Fine and Gray regression analysis stratified by centre was applied to determine the effects of individual covariates; the cumulative incidence of HCC was estimated using the competing risk method with death as a competing risk. Results: A total of 1,428 patients diagnosed with AIH from 1980 to 2020 from 22 eligible centres across Europe and Canada were included, with a median follow-up of 11.1 years (interquartile range 5.2-15.9). Two hundred and ninety-three (20.5%) patients had cirrhosis at diagnosis. During follow-up, 24 patients developed HCC (1.7%), an incidence rate of 1.44 cases/1,000 patient-years; the cumulative incidence of HCC increased over time (0.6% at 5 years, 0.9% at 10 years, 2.7% at 20 years, and 6.6% at 30 years of follow-up). Patients who developed cirrhosis during follow-up had a significantly higher incidence of HCC. The cumulative incidence of HCC was 2.6%, 4.6%, 5.6% and 6.6% at 5, 10, 15, and 20 years after the development of cirrhosis, respectively. Obesity (hazard ratio [HR] 2.94, p = 0.04), cirrhosis (HR 3.17, p = 0.01), and AIH/PSC variant syndrome (HR 5.18, p = 0.007) at baseline were independent risk factors for HCC development. Conclusions: HCC incidence in AIH is low even after cirrhosis development and is associated with risk factors including obesity, cirrhosis, and AIH/PSC variant syndrome Show less
In general the prognosis of treated autoimmune hepatitis is comparable to the general population. The knowledge on risk factors predicting long term survival is still limited. Better knowledge on... Show moreIn general the prognosis of treated autoimmune hepatitis is comparable to the general population. The knowledge on risk factors predicting long term survival is still limited. Better knowledge on risk factors can lead to tailored treatment of patients in the future, preventing side effects in patients currently overtreated and preventing disease progression in patients currently undertreated.At diagnosis age, ethnicity, cirrhosis and ALT level were the main predictors for long-term survival. During treatment ALT level but not IgG level was associated with long-term survival. In patients presenting with acute or acute severe AIH improvement of liver function within 2 weeks after start of treatment was the main predictor for survival. In patients with AIH-PBC variant syndrome more patients than the guidelines indicate are treated with a combination of immunosupression and ursodeoxycholic acid.New treatment options are urgently needed in AIH which requires a better understanding of changes in the immune system. To our suprise complement did not play a role in autoimmune hepatitis. Using B-cell related cytokines, patients could be divided into immunological subgroups with each a different presentation, treatment response and outcome. Show less
