Kanth Rajan, Shrestha Ram Babu, Rai Indira, VanWormer Jeffrey J, Roy Praveen K
At the time the study was conducted, Dr. Kanth was a hospitalist at the Marshfield Clinic, Marshfield, Wisconsin USA. Current affiliation: Carilion Clinic, Department of Gastroenterology, Roanoke, Virginia USA
Integrated Research and Development Laboratory, Marshfield Clinic Research Institute, Marshfield, Wisconsin USA.
Clin Med Res. 2017 Jun;15(1-2):13-18. doi: 10.3121/cmr.2017.1351. Epub 2017 May 9.
Primary biliary cholangitis (PBC) is a rare disease with incidence that varies with time and geography. Only two studies have assessed PBC incidence in the United States, with the most recent appearing over a decade ago. The objective of the present study was to assess PBC incidence in the United States in a more recent era.
The incidence of PBC was assessed in a population-based cohort in rural, Midwestern Wisconsin over two decades spanning from June 1992 through June 2011. Cases were initially identified in the electronic medical record and then manually verified for inclusion according to the American Association for the Study of Liver Disease criteria for PBC. Additional data were abstracted for verified cases.
A total of 79 cases of PBC were identified over the 20-year period for an overall age- and sex-standardized incidence of 4.9 cases per 100,000 person-years. Incidence was higher in females, but changes over time were not significant. After a mean 7.3 years follow-up, all-cause mortality of those with PBC was 29%, and estimated 10-year survival was 76%.
The overall incidence of PBC in a Midwestern population of the United States has remained relatively stable over the last two decades. Patients have better prognosis, and the survival of PBC cases has improved.
原发性胆汁性胆管炎（PBC）是一种罕见疾病，其发病率随时间和地域而变化。仅有两项研究评估了美国的PBC发病率，其中最近的一项研究是在十多年前进行的。本研究的目的是评估美国近期的PBC发病率。
在1992年6月至2011年6月的二十年间，对美国中西部威斯康星州农村地区的一个基于人群的队列进行了PBC发病率评估。病例最初在电子病历中识别，然后根据美国肝病研究协会的PBC标准进行人工核实以纳入研究。对核实的病例提取了其他数据。
在这20年期间共识别出79例PBC病例，总体年龄和性别标准化发病率为每10万人年4.9例。女性发病率较高，但随时间的变化不显著。经过平均7.3年的随访，PBC患者的全因死亡率为29%，估计10年生存率为76%。
在过去二十年中，美国中西部人群中PBC的总体发病率保持相对稳定。患者预后较好，PBC病例的生存率有所提高。
