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Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a long-term progressive liver disease affecting the bile ducts that connect the liver to the gut.

The disease causes bile to build-up, causing cirrhosis (scarring) and damage to the liver.

Because the damage happens over some years, symptoms may not always be noticed to begin with.

Causes of primary biliary cirrhosis

The exact cause of the disease isn’t known, but it is thought to be caused by the body's defence system wrongly attacking the bile ducts, called an autoimmune condition.

Primary biliary cirrhosis is more common in people with other autoimmune conditions, including thyroid problems, rheumatoid arthritis, and coeliac disease.

Up to 20,000 people in the UK have primary biliary cirrhosis. The condition affects women more than it does men, and is usually diagnosed between the ages of 40 and 60.

The condition may have some inherited components, as the risk is higher if a close family member also has primary biliary cirrhosis.

Environmental triggers may also play a role, including infections, smoking, toxic waste and some chemicals.

Primary biliary cirrhosis symptoms

Symptoms of primary biliary cirrhosis include:

Primary biliary cirrhosis diagnosis

Primary biliary cirrhosis diagnosis usually involves blood tests, including those for anti-mitochondrial antibodies (AMAs) and bilirubin levels. The diagnosis may become apparent when someone is being tested for another condition.

Ultrasound scans may be used to check the bile ducts and general health of the liver.

In some cases, a sample of liver tissue will need to be taken for analysis, called a liver biopsy.

Primary biliary cirrhosis treatment

In the earlier stages of primary biliary cirrhosis, medication called ursodeoxycholic acid (UDCA) may be recommended to slow its progression. Other treatment can help relieve specific symptoms, such as itching.

Lifestyle changes may be recommended, such as stopping smoking, weight management and limiting alcohol.

As the condition can lead to liver failure, in some cases a liver transplant may be needed.

A person with primary biliary cirrhosis will be advised to avoid certain medications that are unsuitable if the liver is damaged, including some painkillers.

WebMD Medical Reference

Medically Reviewed by Dr Rob Hicks on September 22, 2017

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