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.

PBC

The cause of inflamed bile ducts in the liver is not known. 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
• Hypothyroidism
• Raynaud's phenomenon
• Sicca syndrome (dry eyes or mouth)

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

Tests for liver dysfunction:

• Serum albumin
• Liver function tests (serum alkaline phosphatase is most important)
• Prothrombin time (PT)
• Serum cholesterol and lipoproteins

Tests for the disease:

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

Therapy aims to relieve symptoms and prevent complications.

Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream and improves the survival of patients with PBC after they 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.

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.

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)

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

Afdhal NH. Diseases of the gall bladder and bile ducts. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier. 2007: chap 159.

Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288.

Silveira MG, Lindor KD. Treatment of primary biliary cirrhosis: therapy with choleretic and immunosuppressive agents. Clin Liver Dis. 2008;12:425-443.