{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T17:41:02Z","timestamp":1776361262333,"version":"3.51.2"},"reference-count":30,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2021,5,21]],"date-time":"2021-05-21T00:00:00Z","timestamp":1621555200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Chang Gung Medical Research Program","award":["CMRPG3I0412, CMRPG3K0721 and CMRPG1K0111"],"award-info":[{"award-number":["CMRPG3I0412, CMRPG3K0721 and CMRPG1K0111"]}]},{"name":"National Science Council","award":["MOST 108-2314-B-182-051-, 109-2314-B-182-024- and 109-2629-B-182-002-"],"award-info":[{"award-number":["MOST 108-2314-B-182-051-, 109-2314-B-182-024- and 109-2629-B-182-002-"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background\/Purpose: The rates and outcomes of primary biliary cholangitis (PBC) in Taiwan remain unclear. Methods: A nationwide population-based cohort study (Taiwan National Health Insurance Research Database, 2002\u20132015) was conducted. Data from four PBC cohorts with various definitions were compared (cohort 1 (C1): ICD-9-CM (571.6); C2: alkaline phosphatase (Alk-P) and antimitochondrial antibody (AMA) measurements; C3: Alk-p and AMA measurements and ursodeoxycholic acid (UDCA) treatment; C4: ICD-9-CM (571.6), Alk-p and AMA measurements and UDCA treatment). Results: The average prevalence rate ranged from 9.419\/105 (C4) to 307.658\/105 (C2), and the female-to-male ratio ranged from 1.192 (C1) to 3.66 (C4). Prevalence rates increased over time in all cohorts. The average incidence rates ranged from 1.456\/105 (C4) to 66.386\/105 (C2). Incidence rates decreased over time in C1 (\u22129.09%, p &lt; 0.0001) and C4 (\u22126.68%, p &lt; 0.0001) and remained steady in the others. C4 had the lowest prevalence and incidence rates and highest female-to-male ratio. Cirrhosis rates ranged from 7.21% (C2) to 39.34% (C4), hepatoma rates ranged from 2.77%(C2) to 6.66%(C1), liver transplantation (LT) rates ranged from 1.07% (C2) to 6.77% (C4), and mortality rates ranged from 18.24% (C2) to 47.36% (C1). C4 had the highest LT (6.77%), osteoporosis (13.87%) and dyslipidemia rates (17.21%). Conclusions: Based on the reported ranges of reasonable rates, female predominance and characteristic outcomes, C4 was the most representative Taiwanese PBC cohort, with average prevalence and incidence rates of 9.419\/105 and 1.456\/105, respectively, and a female-to-male ratio of 3.66. In a 14-year period, cirrhosis, hepatoma, LT, and mortality were noted in 39.34%, 5.52%, 6.77%, and 34.22% of C4 patients, respectively.<\/jats:p>","DOI":"10.3390\/jcm10112226","type":"journal-article","created":{"date-parts":[[2021,5,21]],"date-time":"2021-05-21T13:15:15Z","timestamp":1621602915000},"page":"2226","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Identification of the Representative Primary Biliary Cholangitis Cohort in Taiwan: A Comparison of Four Nationwide Cohorts"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0903-0682","authenticated-orcid":false,"given":"Cheng-Jen","family":"Chen","sequence":"first","affiliation":[{"name":"Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Taoyuan 333423, Taiwan"},{"name":"Department of Medicine, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jur-Shan","family":"Cheng","sequence":"additional","affiliation":[{"name":"Clinical Informatics and Medical Statistics Research Center, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"},{"name":"Department of Emergency Medicine, Chang Gung Memorial Hospital, Keelung 204, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haw-En","family":"Wang","sequence":"additional","affiliation":[{"name":"Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Taoyuan 333423, Taiwan"},{"name":"Department of Medicine, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chun-Wen","family":"Huang","sequence":"additional","affiliation":[{"name":"Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Taoyuan 333423, Taiwan"},{"name":"Department of Medicine, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jing-Hong","family":"Hu","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology, Department of Internal Medicine, Chang Gung Memorial Hospital, Yunlin 638, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8246-0219","authenticated-orcid":false,"given":"Wei-Ting","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Taoyuan 333423, Taiwan"},{"name":"Department of Medicine, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ming-Yu","family":"Chang","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurologic Medicine, Chang Gung Children\u2019s Hospital, Taoyuan 333423, Taiwan"},{"name":"Division of Pediatrics, Chang Gung Memorial Hospital, Keelung 20401, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hsin-Ping","family":"Ku","sequence":"additional","affiliation":[{"name":"Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Taoyuan 333423, Taiwan"},{"name":"Department of Medicine, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cheng-Yu","family":"Lin","sequence":"additional","affiliation":[{"name":"Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Taoyuan 333423, Taiwan"},{"name":"Department of Medicine, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rong-Nan","family":"Chien","sequence":"additional","affiliation":[{"name":"Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Taoyuan 333423, Taiwan"},{"name":"Department of Medicine, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4902-4401","authenticated-orcid":false,"given":"Ming-Ling","family":"Chang","sequence":"additional","affiliation":[{"name":"Division of Hepatology, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, Taoyuan 333423, Taiwan"},{"name":"Department of Medicine, College of Medicine, Chang Gung University, Taoyuan 333323, Taiwan"},{"name":"Liver Research Unit, Department of Gastroenterology and Hepatology, Chang Gung Memorial Hospital, No. 5, Fu Hsing Street, Kuei Shan, Taoyuan 333423, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,5,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1620","DOI":"10.1002\/hep.28140","article-title":"Changing nomenclature for PBC: From \u2018cirrhosis\u2019 to \u2018cholangitis\u2019","volume":"62","author":"Beuers","year":"2015","journal-title":"Hepatology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1002\/hep.28932","article-title":"Long-term clinical impact and cost-effectiveness of obeticholic acid for the treatment of primary biliary cholangitis","volume":"65","author":"Samur","year":"2017","journal-title":"Hepatology"},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"John, B.V., Aitcheson, G., Schwartz, K.B., Shabbir Khakoo, N., Dahman, B., Deng, Y., Goldberg, D., Martin, P., Taddei, T., and Levy, C. (2021). Male Gender is Associated with Higher Rates of Liver-related Mortality in Primary Biliary Cholangitis and Cirrhosis. Hepatology.","DOI":"10.1016\/S0168-8278(20)31408-2"},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"European Association for the Study of the Liver (2017). EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J. Hepatol., 67, 145\u2013172.","DOI":"10.1016\/j.jhep.2017.03.022"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1016\/j.jhep.2009.11.027","article-title":"Primary biliary cirrhosis: A 2010 update","volume":"52","author":"Poupon","year":"2010","journal-title":"J. Hepatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1053\/j.gastro.2014.12.005","article-title":"Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid","volume":"148","author":"Hirschfield","year":"2015","journal-title":"Gastroenterology"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"7577","DOI":"10.1038\/s41598-019-44081-x","article-title":"The extrahepatic events of Asian patients with primary biliary cholangitis: A 30-year cohort study","volume":"9","author":"Lin","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1002\/hep.30174","article-title":"Primary Biliary Cholangitis in Medicare Population: The Impact on Mortality and Resource Use","volume":"69","author":"Sayiner","year":"2019","journal-title":"Hepatology"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1111\/apt.13448","article-title":"Population-based epidemiology of primary biliary cirrhosis in South Korea","volume":"43","author":"Kim","year":"2016","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e116","DOI":"10.1038\/ctg.2017.43","article-title":"Epidemiology and Natural History of Primary Biliary Cholangitis in the Chinese: A Territory-Based Study in Hong Kong between 2000 and 2015","volume":"8","author":"Cheung","year":"2017","journal-title":"Clin. Transl. Gastroenterol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1002\/hep4.1314","article-title":"Burden of Primary Biliary Cholangitis Among Inpatient Population in the United States","volume":"3","author":"Shahab","year":"2019","journal-title":"Hepatol. Commun."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"E664","DOI":"10.9778\/cmajo.20180029","article-title":"Epidemiology and liver transplantation burden of primary biliary cholangitis: A retrospective cohort study","volume":"6","author":"Yoshida","year":"2018","journal-title":"CMAJ Open"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1884","DOI":"10.1002\/hep.23210","article-title":"Epidemiology and natural history of primary biliary cirrhosis in a Canadian health region: A population-based study","volume":"50","author":"Myers","year":"2009","journal-title":"Hepatology"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1136\/gut.42.5.708","article-title":"Ductal dilatation and stenting for residual hepatolithiasis: A promising treatment strategy","volume":"42","author":"Cheng","year":"1998","journal-title":"Gut"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"25906","DOI":"10.1038\/srep25906","article-title":"Evolving Trends in Female to Male Incidence and Male Mortality of Primary Biliary Cholangitis","volume":"6","author":"Lleo","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1338","DOI":"10.1053\/j.gastro.2014.08.029","article-title":"Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: An international follow-up study","volume":"147","author":"Lammers","year":"2014","journal-title":"Gastroenterology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2590","DOI":"10.1111\/liv.14627","article-title":"Primary biliary cholangitis management: Controversies, perspectives and daily practice implications from an expert panel","volume":"40","author":"Alvaro","year":"2020","journal-title":"Liver Int."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/j.gie.2017.02.031","article-title":"Asia-Pacific consensus guidelines for endoscopic management of benign biliary strictures","volume":"86","author":"Hu","year":"2017","journal-title":"Gastrointest. Endosc."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1342","DOI":"10.1016\/j.cgh.2017.12.033","article-title":"Increasing Prevalence of Primary Biliary Cholangitis and Reduced Mortality With Treatment","volume":"16","author":"Lu","year":"2018","journal-title":"Clin. Gastroenterol. Hepatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.jaut.2013.06.015","article-title":"The limitations and hidden gems of the epidemiology of primary biliary cirrhosis","volume":"46","author":"Podda","year":"2013","journal-title":"J. Autoimmun."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1007\/s12072-019-09984-x","article-title":"Epidemiology and clinical course of primary biliary cholangitis in the Asia-Pacific region: A systematic review and meta-analysis","volume":"13","author":"Zeng","year":"2019","journal-title":"Hepatol. Int."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1111\/hepr.13342","article-title":"Increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan","volume":"49","author":"Tanaka","year":"2019","journal-title":"Hepatol. Res."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1093\/aje\/kwt308","article-title":"No rise in incidence but geographical heterogeneity in the occurrence of primary biliary cirrhosis in North East England","volume":"179","author":"McNally","year":"2014","journal-title":"Am. J. Epidemiol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1016\/j.cld.2018.03.011","article-title":"Current Status of Liver Transplantation for Primary Biliary Cholangitis","volume":"22","author":"Aguilar","year":"2018","journal-title":"Clin. Liver Dis."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1093\/ageing\/29.4.305","article-title":"Presentation and mortality of primary biliary cirrhosis in older patients","volume":"29","author":"Newton","year":"2000","journal-title":"Age Ageing"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"e3546","DOI":"10.1097\/MD.0000000000003546","article-title":"Sexual Dimorphic Metabolic Alterations in Hepatitis C Virus-infected Patients: A Community-Based Study in a Hepatitis B\/Hepatitis C Virus Hyperendemic Area","volume":"95","author":"Hu","year":"2016","journal-title":"Medicine"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"2064","DOI":"10.1016\/j.cgh.2020.01.018","article-title":"Hepatitis Flares Are Associated with Better Outcomes Than No Flare in Patients With Decompensated Cirrhosis and Chronic Hepatitis B Virus Infection","volume":"18","author":"Chang","year":"2020","journal-title":"Clin. Gastroenterol. Hepatol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1080\/21505594.2020.1870080","article-title":"Accelerated cardiovascular risk after viral clearance in hepatitis C patients with the NAMPT-rs61330082 TT genotype: An 8-year prospective cohort study","volume":"12","author":"Chang","year":"2021","journal-title":"Virulence"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1501","DOI":"10.1080\/21505594.2020.1838742","article-title":"Altering retinol binding protein 4 levels in hepatitis C: Inflammation and steatosis matter","volume":"11","author":"Chang","year":"2020","journal-title":"Virulence"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"316","DOI":"10.9738\/CC25.1","article-title":"Choledochal cyst originating from primary sclerosing cholangitis in a child","volume":"96","author":"Tseng","year":"2011","journal-title":"Int. Surg."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/10\/11\/2226\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T06:05:14Z","timestamp":1760162714000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/10\/11\/2226"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,21]]},"references-count":30,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2021,6]]}},"alternative-id":["jcm10112226"],"URL":"https:\/\/doi.org\/10.3390\/jcm10112226","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,21]]}}}