{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T14:09:07Z","timestamp":1777039747581,"version":"3.51.4"},"reference-count":23,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":5875,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1999,8]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>There is a widespread impression that the number of patients with the autoimmune liver disease primary biliary cirrhosis (PBC) is increasing, although its incidence and prevalence vary widely. Using thorough case-finding methods and rigorous definitions to assess changes in incidence and prevalence with time and to explore the symptomatology and mortality of the disease in a large group of unselected patients, we performed a descriptive epidemiological study of PBC in a well defined population over a fixed period of time using established diagnostic criteria and with clinical follow-up of all cases. In a population of 2.05 million in northern England 770 definite or probable PBC cases were identified. Prevalence rose from 201.9 per 10<jats:sup>6<\/jats:sup> in the adult population and 541.4 per 10<jats:sup>6<\/jats:sup> women over 40 in 1987 to 334.6 per 10<jats:sup>6<\/jats:sup> adults and 939.8 per 10<jats:sup>6<\/jats:sup> women over 40 in 1994. Incidence was 23 per 10<jats:sup>6<\/jats:sup> in 1987 and 32.2 per 10<jats:sup>6<\/jats:sup> in 1994. Three hundred patients died in median follow-up of 6.27 years (141 liver deaths); the standardized mortality ratio was 2.85. At presumed diagnosis, 60.9% had no symptoms of liver disease. By June 1994 62% of prevalent patients had liver symptoms. PBC is apparently increasing. It is still unclear whether this is because of a true increase, case finding, or increased disease awareness. The study draws attention to (1) high mortality from liver disease and non-liver-related causes even in patients initially with no liver symptoms and (2) apparently poor diagnostic awareness of the disease.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.510300213","type":"journal-article","created":{"date-parts":[[2004,7,28]],"date-time":"2004-07-28T07:26:28Z","timestamp":1090999588000},"page":"390-394","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":184,"title":["Primary biliary cirrhosis once rare, now common in the United Kingdom?"],"prefix":"10.1097","volume":"30","author":[{"given":"Oliver 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