Many people are surprised to find out they have primary biliary cholangitis (PBC). Before their diagnosis of PBC, they might not have heard of this fairly rare disease (previously called “primary biliary cirrhosis”) or known they had liver damage.

People who live with PBC may feel less alone when they talk to others living with the condition. One member of myPBCteam said, “I was told that this was a rare condition, so to be able to talk to others around the world that have it is great.”

Another member, who had been recently diagnosed, said, “I kinda feel like I hit the lottery nobody wants, especially as a male where it’s even more rare, but reading people’s stories here helps a lot.”

Understanding more about primary biliary cholangitis can help, too. Although experts don’t know exactly what causes PBC, they’ve observed who this condition tends to affect. One question people often ask is “How rare is PBC?” If you’ve been wondering about this, here’s the information you need.

Who Is Diagnosed With Primary Biliary Cholangitis?

Primary biliary cholangitis is fairly rare. Globally, about 7 to 940 in a million people have PBC. There’s such a wide range because studies show that the condition is either more common or less common in different groups around the world. Below, we’ll discuss the factors that play a role in which groups of people are more likely to develop PBC.

Gender and Sex

Women are more likely than men to develop primary biliary cholangitis, according to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). When experts discuss how common a condition is in a particular group of people, they often talk about prevalence. This term refers to the number of people in a group who have a specific diagnosis. Prevalence may be given as a percentage (like 50 percent) or a number (like 50 out of 100).

The exact prevalence of PBC varies among studies and sources. A 2014 study reported that about 58 out of every 100,000 women in the United States had PBC, compared with 15 in 100,000 men. In 2024, the American Liver Foundation reported that about 65 out of every 100,000 U.S. women have PBC but didn’t give a prevalence for men.

Studies have found that males may experience PBC differently than females, according to the journal Hepatology Communications, and may be more likely to have a poor prognosis (outlook) with primary biliary cholangitis. Their symptoms might be less likely to respond well to treatment and more likely to progress to something dangerous, like liver failure or end-stage liver disease.

More research is needed to understand exact prevalence rates of PBC.

Age

Primary biliary cholangitis is usually diagnosed when a person is between 45 and 65. That may skew to the older side, as the National Institutes of Health says that people are usually about 60 when they're diagnosed. Other researchers have reported that most women get diagnosed between 30 and 60, according to StatPearls.

However, there are outliers — people younger and older than those age ranges can be diagnosed with PBC. The condition has been diagnosed in people as young as 22 and in people in their 90s. If you think you have symptoms of PBC, you should go to a hepatologist (liver doctor) even if you don’t fit the usual age profile.

Race and Ethnicity

People who are white are more likely to be diagnosed than those of other races, according to the NIDDK. PBC is most common in people whose ancestors came from northern Europe, but it occurs in people of all races from all over the world.

Researchers have reported that it’s possible PBC is diagnosed more frequently in white people because they tend to have better access to health care. Their doctors may also be more likely to know about PBC or treat other people diagnosed with it.

Future research may find that PBC is more common in non-white groups than the data currently shows. More research is necessary to figure out exactly how common PBC is in different racial and ethnic groups.

Family History and Genetics

Primary biliary cholangitis does seem to be influenced by your genes. People who have a parent, brother or sister, or child with it are 11 times more likely to be diagnosed themselves compared to someone who doesn’t have that family history of the disease. Someone who is an identical twin is even more likely to develop the condition if their twin has it. However, daughters of women diagnosed with PBC are at greatest risk, according to StatPearls.

Experts believe that your genetics give you an increased risk of PBC, but something in your environment needs to trigger those genes for you to develop the condition.

Other Diagnoses

Primary biliary cholangitis often occurs with a number of other immune system conditions. These conditions include:

• Sjӧgren’s syndrome
• Raynaud’s phenomenon
• CREST (calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, telangiectasias) syndrome
• Scleroderma
• Lupus
• Autoimmune hepatitis
• Rheumatoid arthritis
• Thyroid conditions
• Connective tissue diseases

The exact prevalence of PBC differs with each of these conditions. In many cases, more research is needed to determine just how commonly they occur together. Researchers also don’t know exactly why having one condition may make it more likely that you develop another. Finally, they don’t know if being diagnosed with PBC can cause the other conditions, if those conditions lead to PBC, or if both are the result of some other underlying health issue.

Urinary Tract Infections

People who’ve had urinary tract infections may be more likely to develop PBC. Once again, more research is needed to fully understand this connection. Researchers think it’s possible that exposure to the infection could trigger PBC.

Environmental Factors

Some environmental factors, including smoking and using hormone replacement therapies, seem to be risk factors for developing primary biliary cholangitis. Being exposed to some toxic chemicals may also be connected to developing PBC.

There may be something about each one of these factors that could affect genes and lead to PBC. Again, more research is needed to understand how common PBC is in people who’ve experienced these exposures and which chemicals might be involved.

Find Your Team

On myPBCteam, the social network for people with primary biliary cholangitis and their loved ones, more than 1,000 members come together to ask questions, give advice, and share their tips with others who understand life with primary biliary cholangitis.

Have you been diagnosed with PBC? Have you been surprised by how rare it is, or do you know others with the same diagnosis? Share your experiences in the comments below, or start a conversation by posting on your Activities page.