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Hormonal and Rare Conditions Primary Biliary Cholangitis What causes PBC?
What causes PBC?
About PBC

What causes PBC?

It is not known what causes PBC, but it is thought to be an autoimmune disease where your immune system (which usually protects you from illness and infection) mistakenly attacks your bile ducts.1,2

It is thought that certain genetic factors combine with environmental factors to trigger this faulty immune response, resulting in PBC:3-5

Two text boxes labelled ‘Genetics’ and ‘Environmental’ with an addition sign in between the two. Genetics: people with a family history of PBC, especially an identical twin who has the disease. Environmental: Infections (urinary or vaginal), smoking, exposure to toxic chemicals, having another autoimmune disease e.g. Sjogren’s syndrome, Raynaud’s phenomenon, Autoimmune thyroid disease.

References

  1. NHS. Primary biliary cholangitis. Available at: https://www.nhs.uk/conditions/primary-biliary-cholangitis-pbc/. Last accessed April 2025.
  2. Mayo Clinic. Primary biliary cholangitis. Available at: https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874. Last accessed April 2025.
  3. Selmi C, Bowlus CL, Gershwin ME, Coppel RL. Primary biliary cirrhosis. Lancet. 2011;377(9777):1600-1609.
  4. Carey EJ, Ali AH, Lindor KD. Primary biliary cirrhosis. The Lancet. 2015;386(10003):1565-1575.
  5. British Liver Trust. Complications and related conditions. Available at: https://britishlivertrust.org.uk/information-and-support/liver-conditions/primary-biliary-cholangitis/complications-and-related-conditions. Last accessed April 2025.