原发性胆汁性肝硬化主要发生于女性，而且与原发性肝癌同时发生较为罕见。尽管原发性肝癌在PBC患者中的发生率很低，但是与其发展相关的几个特点和危险因素已被报道。在本研究中，我们回顾了日本国家数据库中与PBC患者发生癌变相关的近期数据。 通过两项国家调查问卷数据，我们分析了与PBC患者发生肝癌相关的临床病理结果。根据对PBC患者的回顾性数据分析，原发性肝癌的发生率为2.4%。原发性肝癌在男性患者和女性患者中的发生率分别为5.1%和2.0%，男性患者的比例为26.7%。发生癌变的PBC患者与没有HCC的患者相比预后明显较差。通过性别对HCC危险因素进行多变量分析表明：在女性PBC患者中，PBC确诊时的组织学分期可作其进展为HCC的一项独立危险因素，而在男性患者中并非如此。此外，另一项178名PBC发生癌变患者的调查数据显示男性患者PBC与HCC之间诊断时间差明显短于女性患者。
吉林大学第一医院肝病科 张睿 摘译
本文首次发表于[Hepatology,2013,57(5):1942-1949]
Incidence of and risk factors for hepatocellular carcinoma in primary biliary cirrhosis: national data from Japan.
Abstract
Primary biliary cirrhosis (PBC) primarily affects females and is rarely complicated by hepatocellular carcinoma (HCC). Although the HCC incidence in PBC patients is low, several characteristics and risk factors associated with its development have been reported. In this study, national dataconcerning the current status of carcinogenesis in PBC patients in Japan are reviewed. Using data from two national questionnaire surveys, we investigated the clinicopathological findings associated with HCC in PBC patients. According to the data of all reviewed PBC patients, the HCCincidence was 2.4% (71/2946). The HCC incidence by gender was 5.1% (19/370) in males and 2.0% (52/2576) in females, and the proportion of males was 26.7%. Prognosis was significantly poorer in the PBC patients with HCC than in those without. Multivariate analysis of risk factorsassociated with HCC by gender revealed histological stage at the time of PBC diagnosis as an independent risk factor associated with the development of HCC in females, but not in males. Furthermore, data from another national survey of 178 PBC patients with HCC (male/female = 49/129; proportion of males 27.5%) revealed that the duration between the diagnosis of PBC and that of HCC was significantly shorter in males than in females. In addition, histological stage at the time of HCC diagnosis was an independent risk factor for HCC in females, whereas no risk factorswere identified in males. Conclusion: these data indicate that males are at risk of developing HCC at any histological stage of PBC. Therefore, male PBC patients in particular should be carefully screened for HCC from the early stages of PBC.
