Desktop disease specific banner PBC 2x
Hormonal and Rare Conditions Primary Biliary Cholangitis Primary Biliary Cholangitis (PBC)
Primary Biliary Cholangitis (PBC)
About PBC

Primary Biliary Cholangitis (PBC)

Primary biliary cholangitis (previously known as primary biliary cirrhosis) is a rare autoimmune disease that attacks the bile ducts in your liver and gets progressively worse over time.1,2 

PBC gets its name from:2,3

Primary: because the disease itself is the original cause of inflammation, there isn’t any other cause of the inflammation.

Biliary: because it affects the bile ducts.

Cholangitis: because there is inflammation of the bile ducts.

It used to be called primary biliary cirrhosis, but the name was changed because it was a bit misleading. Most people with PBC do not have cirrhosis, which means advanced scarring of the liver.

Image of a liver, gallbladder and hepatic bile duct.

The bile ducts are a series of tubes that transport bile from the liver to the gut. Bile is a fluid made in the liver which is important for digestion, removing waste from the body, and absorbing vitamins.1

PBC causes these tubes to become inflamed and block the passage of bile. Bile builds up in these ducts, which causes further inflammation and can lead to tissue scarring (cirrhosis) and eventually liver failure.2-4

How many people have PBC?

You are much more likely to have PBC if you are female, with 90% of cases being in women.5
The true incidence and prevalence rates of PBC are not known due to varying diagnostic criteria and the quality of local health care across the world.6 The estimated rates can be seen below.7 

Two text boxes labelled ‘Prevalence’ and ‘Incidence’. Prevalence: Worldwide, the number of people with PBC is thought to be between 19 and 402 patients for every 1 million people. Incidence: Every year, between 3 and 58 patients for every 1 million people are diagnosed with PBC, that’s between 27,000-475,000 people around the world

*Based on a worldwide population of 8.2 billion people.

References

  1. NHS. Primary biliary cholangitis. Available at: https://www.nhs.uk/conditions/primary-biliary-cholangitis-pbc/. Last accessed April 2025.
  2. Cleveland Clinic. Primary Biliary Cholangitis (PBC). Available at: https://my.clevelandclinic.org/health/diseases/17715-primary-biliary-cholangitis-pbc. Last accessed April 2025.
  3. Patient.info. Primary biliary cholangitis. Available at: https://patient.info/digestive-health/abnormal-liver-function-tests-leaflet/primary-biliary-cholangitis. Last accessed April 2025.
  4. Mayo Clinic. Primary biliary cholangitis. Available at: https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874. Last accessed April 2025.
  5. Pandit S, Samant H. Primary Biliary Cholangitis. In: StatPearls. Treasure Island (FL): StatPearls Publishing; February 12, 2023.
  6. Selmi C, Bowlus CL, Gershwin ME, Coppel RL. Primary biliary cirrhosis. Lancet. 2011;377(9777):1600-1609.
  7. Orphanet. Primary biliary cholangitis. Available at: https://www.orpha.net/en/disease/detail/186. Last accessed April 2025.