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Primary Biliary Cirrhosis by Diane Stresing

Definition

Primary biliary cirrhosis (PBC) is a chronic inflammation of the bile ducts inside the liver. The irritation and swelling causes blockage of the bile ducts which eventually damages the liver.

Bile Ducts

If you suspect you have PBC, you should 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 doctors to slow the progression of the disease and to treat its symptoms.

Causes

The exact cause or causes of PBC are unknown. However, because 95% of patients have autoantibodies known as antimitochondrial antibodies in their blood, doctors believe it is an autoimmune disease.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition.

Risk factors include:

Gender: Women are nearly ten times as likely as men to develop PBC.
Family History: If someone in your family has PBC, your have a slightly higher risk.
Viral hepatitis: Both hepatitis B and C have also shown to be contributing factors.

If anyone in your family has been diagnosed with PBC, or has other liver problems, or if you have hepatitis B or C, tell your doctor.

Symptoms

If you experience any of these symptoms do not assume it is due to PBC. These symptoms may also be caused by other, less serious health conditions:

Itchy skin
Fatigue
Jaundice (yellowing of the skin and/or eyes)
Abdominal pain
Swelling of the abdomen, especially on the lower right
Joint and skin problems (experienced by some patients)

Diagnosis

Your doctor will ask about your symptoms and medical history, and perform a physical exam.

Tests may include the following:

Blood test—to determine liver enzyme levels in the blood and to look for antimitochondrial antibodies or signs of hepatitis B or C
Endoscopic retrograde cholangiopancreatography (ERCP)—x-ray of the bile ducts to determine the cause and extent of the blockage. To x-ray the bile ducts, a narrow, flexible scope is inserted into the mouth, reaching into the stomach and the small intestine. A thin tube is then placed into the scope to inject dye into the bile ducts so they will be highlighted on the x-ray.

To confirm a diagnosis, your doctor may order a liver biopsy , in which a sample of liver tissue is removed and examined to determine how much damage has occurred.

A liver biopsy is performed under light sedation, usually as an outpatient procedure. A needle is inserted through the skin and into the liver, or through a catheter inserted in a vein and into the liver, to obtain a small tissue sample.

Treatment

Talk with your doctor about the best treatment plan for you. Although there is no known cure for PBC, a variety of treatments may alleviate symptoms, slow the progression of liver damage, and reduce the possibility of complications.

Treatment may include:

Medications

Medications may include:

Cholestyramine ( Questran ) and/or colestipol ( Colestid )—to control itching
Ursodeoxycholic acid ( URSO 250 or Actigall )—can delay the progression of your disease

Vitamin Supplements

Most doctors prescribe, or recommend PBC patients take vitamin supplements, particularly vitamins A, D, K, and calcium, because natural forms of these vitamins cannot be absorbed properly when there is too little bile in the intestines.

Liver Transplant

Once liver damage has become severe, a liver transplant is recommended for patients with PBC.

Prevention

To help reduce your chances of getting PBC, you should maintain good health, especially following a balanced diet to maintain a healthy weight and good cholesterol levels. You should also avoid excessive alcohol to protect your liver’s function.

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:

Biopsy of the liver. Merck Manual Second Home Edition website. Available at: http://www.merck.com/mmhe/sec10/ch134.ch134c.html . Accessed September 21, 2005.
Heathcote JE. Management of primary biliary cirrhosis. Hepatology . 2000;31:1005-1013. Available at: http://www3.interscience.wiley.com/cgi-bin/abstract/106596779/abstract . Accessed September 21, 2005.
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 September 21, 2005.
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.
Worman MD, Howard J. What is primary biliary cirrhosis (PBC)? Columbia University Medical School Gastroenterology Lab website. Available at: http://cpmcnet.columbia.edu/dept/gi/PBC.html . Accessed September 21, 2005.

Last reviewed July 2010 by Purvee S. Shah, MD

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