PBC Basics

Primary biliary cholangitis (PBC) is a lifelong liver disease characterized by bile buildup in the liver causing damage and scarring.

Here are some frequently asked questions (FAQs) about PBC and their answers.

Who gets PBC?

The disease mostly affects women with 90% of patients being women. The exact cause of this is not known. 

Although no direct genetic cause has been identified, it is thought that certain people may have a susceptibility to the disease. Environmental factors could trigger the onset of the disease in such people. 

What causes PBC?

The exact cause of PBC is not known but it is now widely accepted that it is an autoimmune disease in which the body’s own immune system mistakenly attacks the bile ducts causing liver damage.

Can excessive alcohol consumption cause PBC?

PBC is an autoimmune disease caused by an autoimmune attack against the bile ducts. It is not caused by alcohol. In fact, the name of the disease was changed from primary biliary cirrhosis because it was wrongly associated with alcohol misuse. 

However, not regularly drinking alcohol is important to reduce the risk of further liver damage. If you have PBC in the cirrhotic stage, you should completely stop drinking alcohol.

Is PBC contagious?

No, PBC is an autoimmune disease that is neither infectious nor contagious. It cannot be passed from one individual to the other through blood or sex.

What are the early signs of PBC?

Early signs of PBC may include tiredness, itching, stomach pain, nausea, poor appetite and weight loss and fatty deposits under the skin.

How is PBC diagnosed?

Your doctor may diagnose PBC based on blood tests, including liver enzymes tests, the presence of PBC-specific antibodies in your blood, and a liver biopsy.

How common is PBC?

PBC is a rare disease, which is defined as a disease that affects fewer than 200,000 people in the US.

The disease can affect people from all ethnicities. However, the disease is generally more common in Northern Europe and North America. 

Are there any treatments?

There are two main treatments for PBC. These are ursodeoxycholic acid (UDCA) and obeticholic acid (OCA). OCA is usually used in people who cannot tolerate or do not fully respond to UDCA.

There are also new experimental treatments that are currently being investigated for PBC.

In the late stages of the disease, if the damage to the liver is substantial, a liver transplant may be necessary. 

Who will treat me?

If you have PBC, you will be treated by a multidisciplinary team of specialists comprising a hepatologist, pathologist, radiologist and dietitian. In case you need a liver transplant, you will also see a liver surgeon.

What is the outlook like?

This depends on how well you respond to treatment. In some patients, treatment with UDA and/or OCA can normalize the levels of liver enzymes, and these patients may have a normal life expectancy.

With the improvement of patient care and the development of new treatments, the outlook for patients with PBC has improved in recent years.

Patients with PBC who undergo a liver transplant can live up to 20 years after the transplant. However, in about a fifth of the patients, the disease may recur and also affect the new liver. 

Reviewed by Kyle Habet, M.D., on December 15, 2023.