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Primary biliary cirrhosis once rare, now common in the United Kingdom?

Lookup NU author(s): Emeritus Professor Oliver James, Professor Raj Bhopal CBE, Denise Howel, Professor Alastair BurtORCiD


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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 106 in the adult population and 541.4 per 106 women over 40 in 1987 to 334.6 per 106 adults and 939.8 per 106 women over 40 in 1994. Incidence was 23 per 106 in 1987 and 32.2 per 106 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.

Publication metadata

Author(s): James OEW, Bhopal R, Howel D, Gray J, Burt AD, Metcalf JV

Publication type: Article

Publication status: Published

Journal: Hepatology

Year: 1999

Volume: 30

Issue: 2

Pages: 390-394

Print publication date: 01/08/1999

ISSN (print): 0270-9139

ISSN (electronic): 1527-3350

Publisher: John Wiley & Sons, Inc.


DOI: 10.1002/hep.510300213

PubMed id: 10421645


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