Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile. This obstruction damages liver cells and leads to scarring called cirrhosis.A.D.A.M.

Causes, incidence, and risk factors

The cause of inflamed bile ducts in the liver is not known. However, primary biliary cirrhosis is an autoimmune disorder. That means your body's immune system mistakenly attacks healthy tissue.

The disease more commonly affects middle-aged women.

Long-term bile obstruction is believed to lead to liver cirrhosis. The disease may be associated with autoimmune disorders such as:

• Celiac disease
• Raynaud's phenomenon
• Sicca syndrome (dry eyes or mouth)
• Thyroid disease

Symptoms

More than half of patients have no symptoms at the time of diagnosis. Symptoms usually come on gradually and may include:

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

Signs and tests

The doctor or nurse will perform a physical exam.

The following tests can check to see if your liver is working properly:

• Albumin blood test
• Liver function tests (serum alkaline phosphatase is most important)
• Prothrombin time (PT)
• Cholesterol and lipoprotein blood tests

Other tests that can help diagnose this disease are:

• Elevated immunoglobulin M level in the blood
• Liver biopsy
• Anti-mitochondrial antibodies (results are positive in about 95% of cases)

Treatment

The goal of treatment is to ease symptoms and prevent complications.

Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream may improve survival in some patients who have taken it for 4 years.

Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement should be added to prevent or treat soft, weakened bones (osteomalacia).

Liver transplant before liver failure occurs may be successful.

Expectations (prognosis)

The outcome can vary. If the condition is not treated, most patients will need a liver transplant to prevent death from this condition. About a quarter of patients who've had the disease for 10 years will experience liver failure. Doctors can now use a statistical model to predict the best time to do the transplant.

Complications

Progressive cirrhosis can lead to liver failure. Complications can include:

• Bleeding
• Damage to the brain (encephalopathy)
• Fluid and electrolyte imbalance
• Kidney failure
• Malabsorption
• Malnutrition
• Soft bones (osteomalacia)

Calling your health care provider

Call your health care provider if you have:

• Abdominal swelling
• Blood in the stools
• Confusion
• Jaundice
• Itching of the skin that does not go away and is not related to other causes
• Vomiting blood

References

1. Afdhal NH. Diseases of the gallbladder and bile ducts. In:Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 158.
2. Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288. [PubMed: 18456180]

Review Date: 5/1/2012.

Reviewed by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.