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, most commonly with fatigue and itching skin as the first symptom.
Long-standing bile obstruction is believed to lead to liver cirrhosis. The disease may be associated with autoimmune disorders (hypothyroidism, Raynaud's, CREST syndrome, arthridities). There are about 3 to 15 cases per million people in the US population each year.
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