Dataset related to article "Incidence and predictors of hepatocellular carcinoma in patients with autoimmune hepatitis"

This record contains raw data related to article “Incidence and predictors of hepatocellular carcinoma in patients with autoimmune hepatitis" Abstract 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-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. Impact and implications: The risk of developing hepatocellular carcinoma (HCC) in individuals with autoimmune hepatitis (AIH) seems to be lower than for other aetiologies of chronic liver disease. Yet, solid data for this specific patient group remain elusive, given that most of the existing evidence comes from small, single-centre studies. In our study, we found that HCC incidence in patients with AIH is low even after the onset of cirrhosis. Additionally, factors such as advanced age, obesity, cirrhosis, alcohol consumption, and the presence of the AIH/PSC variant syndrome at the time of AIH diagnosis are linked to a higher risk of HCC. Based on these findings, there seems to be merit in adopting a specialized HCC monitoring programme for patients with AIH based on their individual risk factors.本数据集收录与论文《自身免疫性肝炎患者肝细胞癌的发病风险及预测因素》相关的原始数据。 摘要 背景与目的：自身免疫性肝炎（Autoimmune hepatitis, AIH）是一种病因不明的罕见慢性肝病，其肝细胞癌（hepatocellular carcinoma, HCC）发病风险尚未明确，相关危险因素亦未得到充分界定。本研究旨在通过多中心自身免疫性肝炎队列探究肝细胞癌的发病风险，并筛选其预测因素。 方法：本研究针对国际自身免疫性肝炎协作组回顾性登记库中的患者开展一项回顾性观察性多中心研究。评估的临床结局包括肝细胞癌发生、肝移植及死亡。采用按中心分层的Fine-Gray回归分析，以明确各协变量的影响；以死亡作为竞争风险，采用竞争风险模型估算肝细胞癌的累积发病率。 结果：本研究共纳入1980年至2020年间来自欧洲与加拿大22家符合纳入标准的中心、确诊为自身免疫性肝炎的1428例患者，中位随访时间为11.1年（四分位间距5.2~15.9年）。确诊时即存在肝硬化的患者共293例，占比20.5%。随访期间，共24例患者发生肝细胞癌（占比1.7%），发病率为1.44例/1000患者年；肝细胞癌的累积发病率随随访时间延长逐渐升高（随访5年时为0.6%，10年时为0.9%，20年时为2.7%，30年时为6.6%）。随访期间新发肝硬化的患者，其肝细胞癌发病率显著更高。肝硬化发生后5、10、15及20年时，肝细胞癌的累积发病率分别为2.6%、4.6%、5.6%及6.6%。基线时的肥胖（风险比[HR]=2.94，P=0.04）、肝硬化（HR=3.17，P=0.01）及自身免疫性肝炎-原发性硬化性胆管炎（Primary Sclerosing Cholangitis, PSC）重叠综合征（HR=5.18，P=0.007）是肝细胞癌发生的独立危险因素。 结论：即使在合并肝硬化的自身免疫性肝炎患者中，肝细胞癌的发病率仍较低，且与肥胖、肝硬化及自身免疫性肝炎-原发性硬化性胆管炎重叠综合征等危险因素相关。 影响与意义：自身免疫性肝炎患者发生肝细胞癌的风险似乎低于其他病因所致的慢性肝病患者。但由于现有相关证据大多来自小型单中心研究，针对该特定患者群体的可靠数据仍较为匮乏。本研究发现，即使在肝硬化发病后，自身免疫性肝炎患者的肝细胞癌发病率仍较低。此外，确诊自身免疫性肝炎时的高龄、肥胖、肝硬化、饮酒及合并自身免疫性肝炎-原发性硬化性胆管炎重叠综合征，均与肝细胞癌发病风险升高相关。基于上述研究结果，针对自身免疫性肝炎患者制定基于个体危险因素的专属肝细胞癌监测方案，具备一定的临床应用价值。