近日,，國際權(quán)威肝病雜志Hepatology刊登的一則研究"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惡性腫瘤的風(fēng)險顯著增加,。
免疫性因素在腫瘤等惡性疾病的發(fā)生發(fā)展中有多大的影響呢,？新西蘭研究人員近期完成了一項基于人群的，有關(guān)自身免疫性肝病發(fā)病率與其死亡率,、惡性腫瘤發(fā)生率的相關(guān)性研究,。
自身免疫性肝病通常包括：自身免疫性肝炎（AIH）、原發(fā)性膽汁性肝硬化（PBC）,、原發(fā)性硬化性膽管炎（PSC）,。
該研究采用多種方法收集了各種病例，包括私立和公立醫(yī)院,，成人或兒童,，門診及住院病人，實驗室,、放射科及病理科的病例,。所有病例均符合診斷標(biāo)準(zhǔn)。應(yīng)用Kaplan-Meier生存率,，標(biāo)準(zhǔn)死亡率（standardized mortality ratios,，SMR）及標(biāo)準(zhǔn)化發(fā)生率（standard incidence ratios ，SIR）等惡性腫瘤評價標(biāo)準(zhǔn),。
研究共納入130例AIH,、70例PBC和81例PSC患者,。結(jié)果顯示：AIH、PBC和PSC隊列的風(fēng)險率分別為1156人/年,、625人/年和613人/年,，全因死亡SMR分別為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）,，其肝膽管因素死亡SMR分別為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隊列的所有腫瘤SIR分別為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），其肝外腫瘤SIR分別為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，這是首次在同一人群中采用基于人群的研究方法調(diào)查和比較AIH,、PBC和PSC的生存率和腫瘤發(fā)生率,。AIH和PSC患者罹患肝癌及其他惡性腫瘤的風(fēng)險顯著增加。此外,，三個隊列的肝臟相關(guān)死亡率顯著增加,，表明當(dāng)前的治療策略仍不盡如人意。（生物谷Bioon.com）
doi:10.1002/hep.24743
PMC:
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.
