University of Padova, 35122 Padova, Italy.
Scientific Consultant IRCCS Negrar, 37024 Verona, Italy.
Int J Mol Sci. 2024 Feb 12;25(4):2194. doi: 10.3390/ijms25042194.
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterised by the immune-mediated destruction of small and medium intrahepatic bile ducts, with variable outcomes and progression. This review summarises the state of the art regarding the risk of neoplastic progression in PBC patients, with a particular focus on the molecular alterations present in PBC and in hepatocellular carcinoma (HCC), which is the most frequent liver cancer in these patients. Major risk factors are male gender, viral infections, e.g., HBV and HCV, non-response to UDCA, and high alcohol intake, as well as some metabolic-associated factors. Overall, HCC development is significantly more frequent in patients with advanced histological stages, being related to liver cirrhosis. It seems to be of fundamental importance to unravel eventual dysfunctional molecular pathways in PBC patients that may be used as biomarkers for HCC development. In the near future, this will possibly take advantage of artificial intelligence-designed algorithms.
原发性胆汁性胆管炎(PBC)是一种自身免疫性肝病,其特征是小和中型肝内胆管的免疫介导破坏,具有不同的结局和进展。本综述总结了 PBC 患者发生肿瘤进展的风险的最新研究进展,特别关注 PBC 和肝细胞癌(HCC)中存在的分子改变,HCC 是这些患者中最常见的肝癌。主要的危险因素是男性、病毒感染,如 HBV 和 HCV、UDCA 无应答和大量饮酒,以及一些与代谢相关的因素。总的来说,在组织学晚期的患者中 HCC 发生更为频繁,与肝硬化有关。阐明 PBC 患者中可能作为 HCC 发生的生物标志物的潜在功能失调的分子途径似乎至关重要。在不久的将来,这可能会得益于人工智能设计的算法。