PRIMARY BILIARY CIRRHOSIS: Clinical and Associated Autoimmune Features and Natural History
Section snippets
CAUSATION
The most widely accepted hypothesis is that PBC arises in a susceptible individual as a result of exposure to one or more environmental factors. Ninety percent of affected individuals are women,11, 41, 90 but the age and severity of disease at the time of diagnosis are similar in men and women.62 This apparent increase in PBC susceptibility in women mirrors a similar trend in other autoimmune diseases. Associated autoimmune diseases, especially sicca syndrome, are more common in women,62 but as
NATURAL HISTORY
Since the earliest definitive descriptions of PBC, our perception of the condition has changed from being a rare disease with a poor prognosis presenting with jaundice and symptoms of liver failure,89 to being a more common condition often presenting without symptoms following detection through screening with a significantly better prognosis.41, 66 To a considerable extent these changes result from earlier diagnosis leading to a change in our perception of the spectrum of disease. This spectrum
Fatigue
Profound fatigue, which can often adversely affect patient quality of life is an often underdiagnosed symptom of PBC. In an unpublished survey by the United Kingdom-PBC support group (personal communication, 1997) fatigue was voted the worst of all symptoms associated with the disease. Early case series reported fatigue as a symptom in only a small proportion of patients. A demographic study in Ontario, Canada, in which patients reported their own symptoms by questionnaire, reported fatigue to
COMPLICATIONS OF PRIMARY BILIARY CIRRHOSIS
The complications of PBC can be subdivided into metabolic sequelae and complications of cirrhosis.
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Address reprint requests to Margaret F. Bassendine, FRCP, Centre for Liver Research, School of Clinical Medical Sciences, 4th Floor, William Leech Building, The Medical School, Framlington Place, Newcastle upon Tyne, NE2 4HH, United Kingdom, [email protected]
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Centre for Liver Research, The Medical School, University of Newcastle upon Tyne, New Castle upon Tyne, United Kingdom