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Primary Bilary Cirrhosis

Primary biliary cirrhosis is a liver disease that slowly destroys the bile ducts in the liver. Bile, a substance that helps digest fat, leaves the liver through these ducts. When the ducts are damaged, bile builds up in the liver and damages liver tissue. Over time, the disease can cause cirrhosis and may make the liver stop working.

The cause of primary biliary cirrhosis is unknown. The disease affects women more often than men, and usually occurs between the ages of 30 and 60 years. Some research suggests that the disease might be caused by a problem within the immune system.

The most common symptoms of primary biliary cirrhosis are itchy skin and fatigue. Other symptoms include jaundice (yellowing of the eyes and skin), cholesterol deposits on the skin, fluid retention, and dry eyes or mouth. Some people with primary biliary cirrhosis also have osteoporosis, arthritis, and thyroid problems.

Primary biliary cirrhosis is diagnosed through laboratory tests, x rays, and in some cases, a liver biopsy (a simple operation to remove a small piece of liver tissue). Treatment may include taking vitamin and calcium supplements, hormone therapy, and medicines to relieve symptoms. Ursodiol is beneficial for patients with primary biliary cirrhosis although it does not cure the disease. A liver transplant may be necessary if the liver is severely damaged.

National Digestive Diseases Information Clearinghouse
2 Information Way
Bethesda, MD 20892-3570

The National Digestive Diseases Information Clearinghouse (NDDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK is part of the National Institutes of Health under the U.S. Department of Health and Human Services. Established in 1980, the clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. NDDIC answers inquiries, develops and distributes publications, and works closely with professional and patient organizations and Government agencies to coordinate resources about digestive diseases.

Publications produced by the clearinghouse are carefully reviewed by both NIDDK scientists and outside experts.

This e-text is not copyrighted. The clearinghouse encourages users of this e-pub to duplicate and distribute as many copies as desired.

NIH Publication No. 03-4625
March 2003

Also from Medline Plus

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.

Causes, incidence, and risk factors

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 arthridities. There are about 3 to 15 cases per million people in the U.S. population each year.


Enlarged liver
Abdominal pain
Fatty deposits under the skin
Soft yellow spots on the eyelid
Fatty stools
Signs and tests    Return to top

Tests that suggest or confirm the disease:

Liver biopsy
Mitochondrial antibodies (positive in about 95% of cases)
Tests that reveal liver dysfunction:

Liver function tests
Serum cholesterol and lipoproteins may be increased


Therapy is aimed at relief of symptoms and prevention of complications.

Cholestyramine (or colestipol) may reduce the itching by reducing blood levels of bile acids and increasing clearance of bile acids in the skin. Ursodeoxycholic acid may also improve clearance of bile from the bloodstream.

Vitamin replacement therapy restores fat-soluble vitamins A, K, and D lost in fatty stools. A calcium supplement is indicated to prevent osteomalacia.

Periodic evaluation and drainage with ERCP (endoscopic retrograde cholangiopancreatography) or PTC (percutaneous transhepatic cholangiography) may be necessary.

Treatment for episodes of cholangitis may be necessary.

Liver transplant for primary biliary cirrhosis prior to liver failure has been successful.

Expectations (prognosis)

The course is variable, but if the condition is untreated, most patients will need a liver transplant after about seven years. Statistical models are now used to predict the best timing for transplantation.


Progressive cirrhosis can lead to liver failure -- including bleeding, malnutrition, renal failure, fluid, and electrolyte imbalance -- and encephalopathy (damage to the brain). Osteodystrophy (a condition that softens the bones) and malabsorption may develop.
Calling your health care provider    Return to top

Call your health care provider if prolonged itching of the skin develops that is not related to other causes, or if jaundice or other symptoms of cirrhosis develop.

Vomiting blood, blood in the stools, confusion, abdominal swelling, and jaundice are symptoms that indicate cirrhosis may be worsening. Call your provider if these or other new symptoms develop.

Update Date: 5/14/2004

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