Mortality and the risk of malignancy in autoimmune liver diseases: A population-based study in Canterbury, New Zealand
Mortality and the risk of malignancy in autoimmune liver diseases: A population-based study in Canterbury, New Zealand
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DOI：
10.1002/hep.24743
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发表时间：
2012-02-01
期刊：
影响因子：
13.5
通讯作者：
Stedman, Catherine Ann Malcolm
中科院分区：
文献类型：
--
作者：
Ngu, Jing Hieng;Gearry, Richard Blair;Stedman, Catherine Ann Malcolm
关于自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）和原发性硬化性胆管炎（PSC）的死亡率和癌症发病率的基于人群的定量数据很少。我们的目的是系统地调查新西兰坎特伯雷基于人群的 AIH、PBC 和 PSC 队列的生存率和恶性肿瘤风险。采用了多种病例查找方法，包括搜索所有公立和私立、成人和儿科门诊、医院病历、实验室、放射学和病理学报告。符合标准化诊断标准的病例被纳入其中。计算了恶性肿瘤的 Kaplan-Meier 生存估计、标准化死亡率 (SMR) 和标准发病率 (SIR)。共有 130 名 AIH、70 名 PBC 和 81 名 PSC 患者被纳入研究，分别导致 1,156、625 和 613 人年面临风险。对于 AIH、PBC 和 PSC 队列，全因死亡率的 SMR 为 2.1（95% 置信区间 [CI] 1.4-3.1）、2.7（95% CI 1.7-4.0）和 4.1（95% CI 2.6-6.3），肝胆死亡率的 SMR 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)，所有癌症的 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），肝外恶性肿瘤的 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）。结论：这是第一项以人群为基础的研究，旨在检查和比较同一人群中 AIH、PBC 和 PSC 的生存率和癌症发病率。由于肝脏相关死亡，所有三个队列的死亡率均显着增加，表明当前管理策略的不足。 AIH和PSC患者发生肝脏和肝外恶性肿瘤的风险显着增加。 （肝病学 2012）
Population-based quantitative data on the mortality and cancer incidence of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) are 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 1,156, 625, and 613 person-years at risk, respectively. For AIH, PBC, and PSC cohorts, SMRs for all-cause mortality were 2.1 (95% confidence interval [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 extrahepatic 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. Conclusion: 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 extrahepatic malignancy was significantly increased in AIH and PSC patients. (HEPATOLOGY 2012)