Overview

Primary Biliary Cholangitis (PBC) is a chronic, inflammatory autoimmune disease that affects the liver.1,2 It is a rare disease that is most commonly diagnosed in patients in their fifties, but can be diagnosed between the ages of 30 and 70 years old.PBC is characterised by the immune system attacking and gradually destroying the bile ducts.3 As a result, bile acids are poorly eliminated from the bile ducts, which normally take bile from the liver to the gut.2,3 This reduction or stoppage of bile flow is known as cholestasis. The build-up of bile acid in the bile ducts causes damage to the liver.2

Liver diagram

Without treatment, PBC can progress to cirrhosis and eventually lead to liver failure.1,3

Diagnosis

PBC can be identified through blood tests and many patients are diagnosed following a referral from a routine blood test for a different reason.4

Patients are suspected to have PBC if they present with:4,5

  • Elevated alkaline phosphatase (ALP), an enzyme in the bloodstream that is an early indicator of PBC
  • Presence of antimitochondrial antibodies (antibodies that are found in around 95% of patients with PBC)
  • Cholestasis (the reduction or stoppage of bile flow)

Symptoms

Many patients with PBC don’t present with symptoms at time of diagnosis, but may have common symptoms such as fatigue and pruritus.1,5

  • Fatigue is reported in over 50% of patients with PBC and can range from weariness (e.g. struggling to prepare a meal, climb the stairs, perform housework) to complete exhaustion,6 however this can vary considerably from one patient to another.1,7 Any patients with PBC who are experiencing fatigue should discuss their symptoms with their healthcare provider to determine the most appropriate treatment course for them. 
  • Cholestatic pruritus (or itch) is an unpleasant sensation, which leads to the need to scratch.8 It is a major symptom of PBC, affecting up to 70% of patients at some point in their illness.9,10 Pruritus is likely caused by cholestasis, which leads to an increase in concentration of one or more of the substances in the blood that cause pruritus.8 These substances are thought to activate nerve endings, which relay an itch sensation to the brain.10 Any patients experiencing pruritus should discuss their symptoms with their healthcare provider to determine the most appropriate treatment course for them.

It is important to note that there is no correlation between disease activity or progression and the severity of fatigue and pruritus symptoms1

  1. Poupon R. J Hepatol 2010;52:745–58. 
  2. Dyson JK, et al. Nat Rev Gastroenterol Hepatol. 2015;12:147–58. 
  3. Lowth M. Patient, Primary Biliary Cholangitis – Symptoms and treatments. Available at https://patient.info/health/abnormal-liver-function-tests-leaflet/primary-biliary-cholangitis (accessed September 2018).
  4. Hirschfield GM, et al. Gut. 2018;0:1-27.
  5. EASL guidelines. J Hepatol. 2017;67:145-172. 
  6. Cauch-Dudek K et al. Gut. 1998;43:705-710.
  7. Abbas G, et al. Nat. Rev. Gastroenterol. Hepatol. 2010;7:313-319.
  8. Bergasa NV. Journal of Hepatology. 2005;43:1078-1088.
  9. Kaplan MM, et al. N Engl J Med. 2005;353:1261-1273. 
  10. Trivedi HD, et al. The American Journal of Medicine. 2017;130:744.e1-744.e7.