近日，国际权威肝病杂志Hepatology刊登的一则研究"Mortality and the risk of malignancy in autoimmune liver diseases: A population-based study in Canterbury, New Zealand."提示：自身免疫性肝病患者罹患肝癌及其他恶性肿瘤的风险显著增加。
免疫性因素在肿瘤等恶性疾病的发生发展中有多大的影响呢？新西兰研究人员近期完成了一项基于人群的，有关自身免疫性肝病发病率与其死亡率、恶性肿瘤发生率的相关性研究。
自身免疫性肝病通常包括：自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）、原发性硬化性胆管炎（PSC）。
该研究采用多种方法收集了各种病例，包括私立和公立医院，成人或儿童，门诊及住院病人，实验室、放射科及病理科的病例。所有病例均符合诊断标准。应用Kaplan-Meier生存率，标准死亡率（standardized mortality ratios，SMR）及标准化发生率（standard incidence ratios ，SIR）等恶性肿瘤评价标准。
研究共纳入130例AIH、70例PBC和81例PSC患者。结果显示：AIH、PBC和PSC队列的风险率分别为1156人/年、625人/年和613人/年，全因死亡SMR分别为2.1（95%CI：1.4~3.1）、2.7（95%CI：1.7~4.0）和4.1（95%CI：2.6~6.3），其肝胆管因素死亡SMR分别为42.3（95%CI：20.3~77.9）、71.2（95%CI：30.7~140.3）和116.9（95%CI：66.8~189.8）。AIH、PBC和PSC队列的所有肿瘤SIR分别为3.0（95%CI：2.0~4.3）、1.6（95%CI：0.8~2.9）和5.2（95%CI：3.3~7.8），其肝外肿瘤SIR分别为2.7（95%CI：1.8~3.9）、1.6（95%CI：0.8~2.9）和3.0（95%CI：1.6~5.1）。
研究者表示，这是首次在同一人群中采用基于人群的研究方法调查和比较AIH、PBC和PSC的生存率和肿瘤发生率。AIH和PSC患者罹患肝癌及其他恶性肿瘤的风险显著增加。此外，三个队列的肝脏相关死亡率显著增加，表明当前的治疗策略仍不尽如人意。（生物谷Bioon.com）
doi:10.1002/hep.24743
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PMID:
Mortality and the risk of malignancy in autoimmune liver diseases: A population-based study in Canterbury, New Zealand.
Ngu JH, Gearry RB, Frampton CM, Stedman CA.
Population-based quantitative data on the mortality and cancer incidence of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) is scarce. Our aim was to systematically investigate the survival and risk of malignancy on population-based cohorts of AIH, PBC and PSC in Canterbury, New Zealand. Multiple case finding methods were employed, including searches of all public and private, adult and pediatric outpatient clinics, hospital notes, laboratory, radiology and pathology reports. Cases that fulfilled standardized diagnostic criteria were included. Kaplan-Meier survival estimates, standardized mortality ratios (SMR) and standard incidence ratios (SIR) for malignancy were calculated. A total of 130 AIH, 70 PBC and 81 PSC patients were included contributing to 1156, 625 and 613 person-year at risk, respectively. For AIH, PBC and PSC cohorts, SMRs for all-cause mortality were 2.1 (95% CI 1.4-3.1), 2.7 (95% CI 1.7-4.0) and 4.1 (95% CI 2.6-6.3), SMRs for hepatobiliary mortality were 42.3 (95% CI 20.3-77.9), 71.2 (95% CI 30.7-140.3) and 116.9 (95% CI 66.8-189.8), SIRs for all cancers were 3.0 (95% CI 2.0-4.3), 1.6 (95% CI 0.8-2.9) and 5.2 (95% CI 3.3-7.8), and SIRs for extra-hepatic malignancy were 2.7 (95% CI 1.8-3.9), 1.6 (95% CI 0.8-2.9) and 3.0 (95% CI 1.6-5.1) respectively. CONCLUSIONS: This is the first population-based study to examine and compare the survival and cancer incidence in AIH, PBC and PSC in the same population. The mortality for all three cohorts was significantly increased due to liver related death demonstrating the inadequacy of current management strategies. The risk of hepatic and extra-hepatic malignancy was significantly increased in AIH and PSC patients.
