Primary Biliary Cirrhosis
(PBC)
Definition
Primary biliary cirrhosis (PBC) is a chronic condition inside the liver. It is swelling in a part of the liver called bile ducts. Overtime this swelling can cause permanent damage to the bile ducts.
The liver creates a fluid called bile. The fluid is sent out of the liver through bile ducts. The bile then moves to the gallbladder, and finally the small intestine. Bile helps break down food in the intestines. PBC makes it difficult for bile to move out of the liver. The bile is not able to pass through the damaged bile ducts. As a result the bile backs up into the liver. This ultimately leads to liver damage.
Bile Ducts
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If you suspect you have PBC, work with your doctor to begin treatment as soon as possible. Most people with PBC live full lives for many years after being diagnosed. The long-term prognosis is best for those who work closely with their doctor.
Causes
The exact cause or causes of PBC are unknown. However, because 95% of patients have specific autoantibodies. This may indicate an autoimmune disorder. This means the immune system is attacking health tissue instead of foreign items like germs.
Risk Factors
Factors that increase your chance of PBC include:
- Gender: Women are nearly ten times as likely as men to develop PBC
- Family History
- Viral hepatitis (both hepatitis B and C)
Symptoms
Symptoms of PBC include:
- Fatigue
- Itchy skin
- Abdominal pain, especially in right upper abdomen
- Signs of liver damage:
- Jaundice (yellowing of the skin and/or eyes)
- Spider veins
- Xanthelasma—yellow deposits around eyelids
Diagnosis
Your doctor will ask about your symptoms and medical history. A physical exam will be done. Blood tests will help to determine the extent of liver problems. They may also help look for causes like a hepatitis infection or autoimmune disorder. A liver biopsy will also help determine how much liver damage has occurred.
Detailed pictures of the bile ducts may be needed. To get these pictures, your doctor may order:
- MRI scan
- CT scan
- Ultrasound
- Endoscopic retrograde cholangiopancreatography (ERCP)
Treatment
There is no known cure for PBC. However, a variety of treatments may help to manage symptoms. Treatment can also help to slow the progression of liver damage and reduce the possibility of complications.
Talk with your doctor about the best treatment plan for you. Treatment options may include:
Medications
Certain medications may help control itching. Ursodeoxycholic acid may also help to move bile through the bile ducts.
Nutrition
Your doctor may recommend vitamin supplements. The low levels of bile may make it difficult for your body to breakdown food. As a result you can not get enough vitamins from food. Vitamins A, D, K, and calcium are commonly recommended.
A healthy, well-balanced diet helps your overall health. Your doctor may recommend supplements if you are having trouble reaching your nutrition goals. You should also avoid raw shellfish if you have cirrhosis.
Avoid alcohol or other items that can effect your liver. Talk to your doctor about any medications you are taking. Even some over-the-counter medications can be harmful with cirrhosis.
Liver Transplant
A liver transplant is the only complete cure for PBC. It is only considered when other treatments are unable to control symptoms.
RESOURCES:
American Liver Foundation
http://www.liverfoundation.org
National Institute of Diabetes & Digestive & Kidney Diseases
http://www.digestive.niddk.nih.gov
Primary Biliary Cirrhosis Organization
http://www.pbcers.org
CANADIAN RESOURCES:
Canadian Digestive Health Foundation
http://www.cdhf.ca
Canadian Liver Foundation
http://www.liver.ca
REFERENCES:
Heathcote JE. Management of primary biliary cirrhosis. Hepatology . 2000;31:1005-1013. Available at: http://www3.inters... . Accessed December 11, 2012.
Lindor KD, Gershwin ME, Poupon R, et al. Primary biliary cirrhosis. Hepatology . 2009; 50:291.
Metcalf JV, Howel D, James OFW, Bhopal R. Primary biliary cirrhosis: epidemiology helping the clinician. Br Med J . 1996;312:1181-1182. Available at: http://bmj.bmjjournals.com/cgi/content/full/312/7040/1181 . Accessed December 11, 2012.
Poupon RE, Balkau B, Eschwege E, Poupon R. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-BPC Study Group. N Engl J Med . 1991; 324:1548-1554.
Primary biliary cirrhosis. American Liver Foundation website. Available at: http://www.liverfoundation.org/abouttheliver/info/pbc/. Updated April 30, 2012. Accessed December 11, 2012.
Primary biliary cirrhosis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php. Updated February 29, 2012. Accessed December 11, 2012.
Primary biliary cirrhosis. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) website. Available at: http://digestive.n.... Updated April 30, 2012. Accessed December 11, 2012.
Last reviewed December 2012 by Michael Woods, MD
Last Updated: 12/11/2012