Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a chronic and progressive disease of unknown aetiology leading to destruction of the small-to-medium bile ducts, progressive cholestasis and often end-stage liver disease. Fatigue is the first reported symptom with pruritus a very disturbing symptom. Investigations show significant elevations of ALP, GGTP and IgM. After the patient has become symptomatic, life-expectancy is 5 to 10 year. Immunosuppressive agents inhibit immune reactions that mediate the progression of the disease.

 

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