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What is Primary Biliary Cholangitis (PBC)?

Also known as Primary Biliary Cirrhosis, PBC is a rare condition which affects some of the biliary structures in the body, specifically those within the liver. The biliary structures are key to the transport of bile, a substance that is manufactured in the liver and is important in digestion. Gradually, these ducts break down. The bile then builds up in the liver, causing inflammation and damage. 

 

Who typically gets PBC? 

Currently, PBC is believed to be autoimmune. An autoimmune condition is where the body mistakes its own cells for external cells and so attacks itself, often caused by an individual’s genetics. Most people with PBC also have the presence of Anti-Mitchondrial Antibodies (AMAs) which attack the biliary cells in the liver. It is common that sufferers have other autoimmune conditions in addition to PBC.  

What are the symptoms of PBC?

The biliary structures which are progressively damaged in PBC, help transport bile to where it is required to aid digestion. Therefore, when they are damaged, vitamin deficiency is a common outcome as well as digestion issues. Your doctor may use the word Cholestasis to refer to this obstructed bile flow due to PSC.  

Other frequent symptoms include tiredness, dry eyes and mouth, abdominal pain and jaundice. Bile build up in the liver causes damage to the liver cells and so its functioning. It’s possible that this damage could lead to cirrhosis and the need for a transplant. 

How is PBC diagnosed? 

PBC is currently diagnosed via a mixture of bloods, imaging and biopsy. Blood tests would at first look for indicators particularly related to the health of the biliary structures such as ALP (Alkaline Phosphatase) and Albumin. However, there would be other indicators as part of a normal liver blood test that would interest your doctor such as AST and ALT. In addition, it is normal for a blood test to detect the presence of AMA (Anti-Mitochondrial Antibodies) to be taken. The vast majority of people with PBC have high levels of AMAs, molecules which target the biliary cells causing breakdown.  

Imaging tests such as an MRI of the biliary tree called an MRCP (Magnetic Resonance Cholangiopancreatography) may also be taken. A liver biopsy may also be necessary both for diagnosis and ongoing monitoring of the condition.  

What is the treatment for PBC?

Treatment for PBC can involve symptom management and condition management. You can ask your doctor about the various treatments available for symptoms such as itching. The condition is most commonly managed via use of ursodeoxycholic acid, a bile acid, which is thought to help the flow of bile out of the liver and so slow down the gradual damage that having PBC can cause. Please ask your medical team about the full range of options available for management of PBC

Are there any PBC support groups I can contact? 

For more information about PBC visit UK PBC and for patient support visit the PBC Foundation.