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Primary biliary cirrhosis is a chronic, progressive, cholestatic liver disease
which classically affects middle aged women.
- this disease is uncommon (about 30 cases per million) and found mainly in
women (95 per cent of sufferers)
- primary biliary cirrhosis is rare under the age of 30 - most commonly
occurring between 30 and 65 years of age
- end stage disease results in liver failure and the need for liver transplantation
- patients probably have a genetic predisposition but require environmental
triggers to initiate the disease.
- primary biliary cirrhosis is characterised by immune dysfunction; 95% of
patients have anti-mitochondrial autoantibodies
- diagnosis should be suspected in a patient particularly a woman who
presents with fatigue, itching, jaundice or right upper quadrant discomfort
(1)
Reference:
- Prescriber 2003; 14(23):20-9.
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