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Primary Biliary Cirrhosis

From Mediwikis
Autoimmune disease causing progressive destruction of bile canniculi (intra hepatic bile ducts) within the liver. Bile builds up damaging the liver, leading to cirrhosis and cholestatic jaundice.
Remember the "M rule": IgM, AMA, Middle aged females

Epidemiology

  • Most common autoimmune liver disease
  • M:F ratio is 1:10
  • Disease of middle age

Presentation[1]

  • Fatigue/ daytime sleepiness
  • Jaundice
  • Pruritus
  • Xanthoma
  • Fluid retention

In later stages of disease cirrhosis develops, leading to portal hypertension, and Splenomegaly & Oesophageal varices.

Investigations

  • AMA +ve (anti-mitochondrial antibody) – in >90% of cases
  • ANA +ve (anti nuclear antibodies) – common, but not very specific, can be used as prognostic agent as some specific types make it more likely to progress to portal hypertension and liver failure.
  • LFTs – have a cholestatic picture (raised ALP and gamma GT) and a high cholesterol
  • USS/CT to rule out other causes of symptoms such as stones or strictures
  • Liver biopsy – to determine stage

Management

  • Ursodeoxycholic acid- improves bile flow
  • Cholestyramine- removes bile acids in the blood to relieve itch
  • Vitamin supplementation (KADE)
  • No alcohol in diet
  • Liver transplant

References

  1. http://www.britishlivertrust.org.uk/liver-information/liver-conditions/primary-biliary-cirrhosis/