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Primary biliary cirrhosis is an inflammation (irritation and swelling) of the bile ducts of the liver resulting in narrowing and obstruction of the flow of bile . This obstruction damages liver cells.
The cause of inflamed bile ducts within the liver in this condition is not known. The disease more commonly affects middle-aged women. The onset of symptoms is gradual, with fatigue and itching skin as the most common first symptom.
Long-standing bile obstruction is believed to lead to liver cirrhosis . The disease may be associated with autoimmune disorders such as hypothyroidism, Raynaud's, CREST syndrome, and arthritides. There are about 3 to 15 cases per million people in the U.S. population each year.
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