Selmi Carlo, Gershwin M Eric, Lindor Keith D, Worman Howard J, Gold Ellen B, Watnik Mitchell, Utts Jessica, Invernizzi Pietro, Kaplan Marshall M, Vierling John M, Bowlus Christopher L, Silveira Marina G, Bossi Ilaria
Division of Rheumatology, Allergy, and Clinical Immunology, University of California, Davis, CA 95616, USA.
Hepatology. 2007 Dec;46(6):1836-43. doi: 10.1002/hep.21953.
Primary biliary cirrhosis (PBC) is generally a slowly progressive disease that may lead to cirrhosis and liver failure. However, patients with PBC often suffer from a variety of symptoms long before the development of cirrhosis that include issues of daily living that have an impact on their work environment and their individual quality of life. We therefore examined multiple parameters by taking advantage of the database of our cohort of 1032 patients with PBC and 1041 matched controls. The data were obtained from patients from 23 tertiary referral centers throughout the United States and from rigorously matched controls by age, sex, ethnicity, and random-digit dialing. The data showed that patients with PBC were more likely than controls to have significant articular symptoms, a reduced ability to perform household chores, and the need for help with routine activities. Patients with PBC rated their overall activity similar or superior to that of controls; however, more of them reported limitations in their ability to carry out activities at work or at home and difficulties in everyday activities. PBC cases also more frequently reported limitations in participating in certain sports or exercises and pursuing various hobbies; however, they did not report significant limitations in social activities. In a multivariable analysis, household income, a diagnosis of systemic lupus erythematosus, limitations in work activities, a reduction in work secondary to disability, and church attendance were independently increased in PBC cases with respect to controls.
Our data indicate that the quality of life of patients with PBC in the United States is generally well preserved. Nevertheless, patients with PBC suffer significantly more than controls from a variety of symptoms that are beyond the immediate impact of liver failure and affect their lifestyle, personal relationships, and work activities.
原发性胆汁性肝硬化（PBC）通常是一种进展缓慢的疾病，可能导致肝硬化和肝衰竭。然而，PBC患者在肝硬化发展之前往往就会出现各种症状，包括影响其工作环境和个人生活质量的日常生活问题。因此，我们利用我们队列中1032例PBC患者和1041例匹配对照的数据库，对多个参数进行了研究。数据来自美国23个三级转诊中心的患者以及通过年龄、性别、种族和随机数字拨号严格匹配的对照。数据显示，PBC患者比对照更有可能出现明显的关节症状、做家务能力下降以及在日常活动中需要帮助。PBC患者对其总体活动的评价与对照相似或更好；然而，更多患者报告在工作或家中开展活动的能力受限以及日常活动存在困难。PBC患者也更频繁地报告在参与某些运动或锻炼以及从事各种爱好方面存在限制；然而，他们并未报告在社交活动方面有明显限制。在多变量分析中，与对照相比，PBC患者的家庭收入、系统性红斑狼疮诊断、工作活动受限、因残疾导致的工作减少以及参加教堂活动独立增加。
我们的数据表明，美国PBC患者的生活质量总体上得到了较好的保留。然而，PBC患者比对照更容易出现各种症状，这些症状超出了肝衰竭的直接影响，影响了他们的生活方式、人际关系和工作活动。
