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Disease pathophysiology:
Primary biliary cirrhosis is inflammation with progressive scarring of the bile ducts in the liver. Eventually, the ducts are blocked, the liver becomes scarred, and cirrhosis and liver failure develop. Primary biliary cirrhosis probably results from an autoimmune reaction. This disorder commonly causes itchiness, fatigue, dry mouth and eyes, and jaundice, but some people have no symptoms.
Disease statistics:
The prevalence of PBC has been estimated as 12.9 per 100,000 population with up to 90% of cases occurring in women. Diagnosis is usually between about 45 and 55 years old.The prevalence appears to have been rising since 1980. This may be in part due to increased survival but is probably also due to better awareness of the disease.
Disease treatment:
The goals of treatment are to slow the progression rate of the disease and to alleviate the symptoms (eg, pruritus, osteoporosis, sicca syndrome). Liver transplantation appears to be the only life-saving procedure. Immunosuppressive agents inhibit immune reactions that mediate the progression of the disease. Increased activity is recommended, especially in postmenopausal women, to prevent osteoporosis.
