Primary biliary cirrhosis

Definition

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.

Alternative Names

PBC

Causes, incidence, and risk factors

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:

• Arthritis
• CREST syndrome
• Hypothyroidism
• Raynaud's phenomenon

Symptoms

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

Tests for the disease:

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

Tests for liver dysfunction:

• Liver function tests
• Serum cholesterol and lipoproteins

Treatment

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.

Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement can help prevent 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 after about 7 years. Doctors can now use statistics 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 (osteodystrophy)

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

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

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