Primary Biliary Cholangitis

Primary biliary cholangitis (PBC) is a chronic condition that results in the destruction of bile ducts within the liver.

your quick guide to this page

what is primary biliary cholangitis?: Learn what defines primary biliary cholangitis and what its general consequences are.
fast facts: Get a quick overview of primary biliary cholangitis through statistics and bite-sized facts.
symptoms: Learn about the symptoms of primary biliary cholangitis, how to recognize them, and what to do if you experience them.
lifestyle impacts: Find out how primary biliary cholangitis impacts your lifestyle.
what can I do?: Discover management and treatment strategies for primary biliary cholangitis.
additional resources: Find useful links, education, articles, recipes and more that can help with primary biliary cholangitis.

what is primary biliary cholangitis?

Primary biliary cholangitis, formerly known as primary biliary cirrhosis, is a chronic liver disease. When a person has PBC, the immune system attacks the liver causing slow, progressive damage to the bile ducts (small tubes through which bile flows out of the liver).

What is Bile?

Bile is a yellow-green fluid produced by your liver to aid digestion. When the bile ducts are damaged, bile and other substances can accumulate in the liver causing inflammation and liver damage. Over time this can result in cirrhosis (scarring of the liver).

symptoms

Patients with primary biliary cholangitis often do not experience any symptoms. However, when symptoms are present they include:

Jaundice (yellowing of the skin or eyes)
Mild to disabling chronic fatigue
Mild to intense and unrelenting itching of the skin (often on palms or soles of the feet)
Swelling of the legs and feet (edema)
Small white bumps under the skin, usually around the eyes
Dry mouth and eyes
Bone, muscle and joint pain

lifestyle impacts

Many patients are able to live a normal life with PBC since they experience little to no symptoms. When patients do experience symptoms, they can have a significant impact on their daily activities. Chronic fatigue is a common symptom of PBC which may make it difficult for patients to work and maintain a social life.

There are many lifestyle changes that can be done to help manage PBC symptoms and make you feel better. These changes include:

Consuming a balanced diet low in fats and processed sugars
Drink plenty of water
Avoid or lower intake of alcohol
Consistent physical activity
Stop smoking

what can I do?

prevention

The cause of PBC is unknown but research suggests it may be a result of problems with the immune system. Genetic factors may also play a role in developing this disease.

diagnosis

Since many patients with PBC do not experience any symptoms, diagnosis is often found through routine blood tests. For patients that are experiencing symptoms, a blood test is done to check the levels of antimitochondrial antibodies in the blood. If this test comes back positive, it is likely that the patient has primary biliary cholangitis. A liver biopsy (small sample of liver tissue) may also be requested to confirm the diagnosis and rule our other diseases. A liver biopsy, where a small sample of liver tissue is removed with a small needle can help confirm the diagnosis.

treatment

PBC is a chronic long-lasting condition that can be controlled in most patients, but not cured. The prognosis of PBC has improved over the last two decades due to earlier diagnosis and improved treatment. Early access to treatment can significantly delay progression of the disease.

A medication called ursodeoxycholic acid (also called UDCA, URSO) mimics a naturally occurring bile acid. This medication can improve liver function and delay the development of fibrosis (scar tissue) in the liver, which in turn can delay or eliminate the potential for PBC to progress towards liver failure and/or liver transplantation.
Vitamins A, D, E and K can be prescribed, usually when severe cholestasis and jaundice is present.
Various medications may be prescribed by your doctor to help reduce itching. Some examples of these medications include cholestyramine, antihistamines or other medications.
Obeticholic acid (OCA), which is a man-made bile acid that acts to reduce liver inflammation and cholestasis, is available in Canada.

Liver transplantation may be recommended for eligible candidates if other treatments are no longer helpful, and liver damage and scarring has progressed to the point that the liver is no longer able to work properly. Liver transplantation works well for people with PBC, although it is possible to develop PBC in your new liver.