Primary biliary cholangitis

Last revised by Joshua Yap on 21 Oct 2022

Primary biliary cholangitis (PBC) is a chronic progressive cholestatic liver disease that is the cause of 1-2% of deaths from cirrhosis and constitutes the third most common indication for liver transplantation in adults.

The name of this disease was changed from primary biliary cirrhosis to primary biliary cholangitis in 2014; however, it was also decided to keep the abbreviation as PBC 11.

The typical patient is a middle-aged woman presenting with symptoms of fatigue and pruritus and laboratory test evidence of cholestasis.

Clinical features include 12:

  • pruritus (often the first symptom)

  • jaundice

  • skin hyperpigmentation, especially of arms and trunk

  • xanthomas and xanthelasma due to hypercholesterolemia

    • interestingly hypercholesterolemia does not confer an increased risk of death from atherosclerosis

  • digital clubbing

  • fatigue

  • right upper quadrant abdominal discomfort

Primary biliary cholangitis is characterized by the destruction of small intrahepatic bile ducts, portal inflammation, and progressive scarring. The cause of primary biliary cholangitis is unknown, but it is probably due to an inherited abnormality of immunoregulation.

  • T2: if parenchymal lace-like fibrosis and periportal halo sign are seen together the sensitivity for primary biliary cholangitis can approach 70% 2

Other features detectable on MRI include:

  • periportal hyperintensity (cuffing)

  • segmental hypertrophy, notably of the caudate lobe

  • hepatic surface irregularity due to regenerative nodules

  • regional lymphadenopathy: tends to dominate in the gastrohepatic ligament and porta hepatis 4

  • splenomegaly

Ursodeoxycholic acid is the mainstay for medical management 12. In cases of primary biliary cholangitis that advance despite pharmacotherapy, liver transplantation clearly improves the survival and quality of life.

Primary biliary cholangitis can recur after transplantation but with a frequency much lower than that for hepatitis and other diseases.

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