Primary biliary cirrhosis (PBC) is a chronic condition inside the liver. It is swelling in a part of the liver called bile ducts. Over time, 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 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 leads to liver damage.
The exact cause of PBC is unknown. It appears to be an autoimmune disorder. This means the immune system is attacking health tissue. It is not known what causes an autoimmune reaction. It is likely due to a combination of genetic and environmental factors.
PBC is more common in women. Other factors that may increase the chances of PBC:
- Family history
- Viral hepatitis—both hepatitis B and C
Symptoms of PBC include:
- Itchy skin
- Abdominal pain, especially in right upper abdomen
Signs of liver damage:
- Yellowing of the skin and/or eyes— jaundice
- Spider veins
- Xanthelasma—yellow deposits around eyelids
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
- Endoscopic retrograde cholangiopancreatography (ERCP)
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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:
Certain medications may help control itching. Ursodeoxycholic acid may also help to move bile through the bile ducts.
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 cannot 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 affect your liver. Talk to your doctor about any medications you are taking. Even some over-the-counter medications can be harmful with cirrhosis.
A liver transplant is the only complete cure for PBC. It is only considered when other treatments are unable to control symptoms.
American Liver Foundation https://www.liverfoundation.org
National Institute of Diabetes and Digestive and Kidney Diseases https://www.niddk.nih.gov
Canadian Liver Foundation https://www.liver.ca
Health Canada https://www.canada.ca
Carey EJ, Ali AH, Lindor KD. Primary biliary cirrhosis. Lancet. 2015;386(100030:1565-1575.
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(22):1548-1554.
Primary biliary cholangitis (PBC). EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T114824/Primary-biliary-cholangitis-PBC . Updated October 11, 2017. Accessed April 4, 2018.
Primary biliary cirrhosis. American Liver Foundation website. Available at: https://www.liverfoundation.org/for-patients/about-the-liver/diseases-of-the-liver/primary-biliary-cholangitis. April 4, 2018.
Primary biliary cirrhosis. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at: https://www.niddk.nih.gov/health-information/liver-disease/primary-biliary-cholangitis. Accessed April 4, 2018.
- Reviewer: EBSCO Medical Review Board Daus Mahnke, MD
- Review Date: 03/2018
- Update Date: 05/08/2014