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.

• Itching
• Jaundice
• Enlarged liver
• Abdominal pain
• Fatty deposits under the skin
• Soft yellow spots on the eyelid
• Fatty stools

Tests that suggest or confirm the disease:

• Liver biopsy
• Mitochondrial antibodies (positive in about 95% of cases)
• Elevated immunoglobulin M level in the blood

Tests that reveal liver dysfunction:

• Liver function tests
• Serum cholesterol and lipoproteins may be increased