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Primary biliary cirrhosis
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Alternative Names Return to top
PBCDefinition Return to top
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.
Causes Return to top
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 Return to top
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
Exams and Tests Return to top
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 Return to top
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.
Outlook (Prognosis) Return to top
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.
Possible Complications Return to top
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)
When to Contact a Medical Professional Return to top
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 Return to top
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.
Update Date: 5/20/2008 Updated by: Christian Stone, MD, Division of Gastroenterology, Washington University in St. Louis School of Medicine, St. Louis, MO. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 1997-2009, A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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