Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: Follow-up for up to 28 years
Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: Follow-up for up to 28 years
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DOI：
10.1053/gast.2002.36027
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发表时间：
2002-10-01
期刊：
影响因子：
29.4
通讯作者：
James, OFW
中科院分区：
文献类型：
--
作者：
Prince, M;Chetwynd, A;James, OFW
背景和目标：虽然一些优秀的研究已经描述了原发性胆汁性肝硬化的自然史，但大多数报告来自三级转诊中心。我们在一个综合性的地理学定义的队列中研究了原发性胆汁性肝硬化的预后。研究方法：我们随访了770例原发性胆汁性肝硬化患者，流行于1987年1月至1994年12月，直到2000年1月1日死亡，移植，或审查，通过采访和审查的情况下，笔记和死亡证明。生存数据采用Kaplan-Meier法和考克斯回归分析。结果：从诊断开始，患者的中位生存期为9.3年。在考克斯模型中，患者年龄、诊断时的碱性磷酸酶、白蛋白和胆红素独立预测生存率。凝血酶原时间和组织学分期并不独立影响生存率。通过该模型和马约预后评分（R-M（2）分别= 0.37和0.18; R-M（2）是由于协变量而从模型获得的百分比信息的基于似然性的测量），可以很好地预测观察到的生存期。42%的死亡是由肝病引起的。截至删失日期，39例患者接受了肝移植。生存率比年龄和性别匹配的对照人群差得多（标准化死亡率= 2.87 [1.73，不包括肝脏死亡]）。诊断时最常见的症状是瘙痒（18.9%）和疲劳（21.0%）。26%的患者在诊断后10年内出现肝功能衰竭。结论：虽然原发性胆汁性肝硬化现在通常在早期诊断，但诊断仍然具有重要的预后意义。很大一部分患者在诊断后发生肝功能衰竭，需要移植，或过早死亡。
Background & Aims: Although several excellent studies have described the natural history of primary biliary cirrhosis, most were reported from tertiary referral centers. We examined the prognosis of primary billary cirrhosis in a comprehensive geographically defined cohort. Methods: We followed up 770 primary biliary cirrhosis patients prevalent between January 1987 and December 1994 until death, transplantation, or censor on January 1, 2000, by interview and review of case notes and death certificates. Analysis of survival data was performed with Kaplan-Meier methods and Cox regression. Results: Median patient survival was 9.3 years from diagnosis. Patient age, alkaline phosphatase, albumin, and bilirubin at diagnosis independently predicted survival in Cox modeling. Prothrombin time and histologic stage did not independently affect survival. Observed survival was predicted well by this model and by the Mayo prognostic score (R-M(2) = 0.37 and 0.18, respectively; R-M(2) is a likelihood-based measure of the percentage information gain from the model due to covariates). Forty-two percent of deaths were caused by liver disease. Thirty-nine patients had liver transplantations by the censor date. Survival was much poorer than for an age- and sex-matched control population (standardized mortality ratio = 2.87 [1.73 excluding liver deaths]). The most common symptoms at diagnosis were pruritus (18.9%) and fatigue (21.0%). Twenty-six percent of patients developed liver failure by 10 years after diagnosis. Conclusions: Although primary biliary cirrhosis is often now diagnosed at an early stage, the diagnosis still carries important prognostic implications. A significant proportion of patients develop liver failure, require transplantation, or die prematurely after this diagnosis.