Primary billiary cirrhosis

Question 1

Define primary billiary cirrhosis

Answer 1

Damage and remodelling of the intrahepatic billiary ducts
Progressive, and the complications only really arrive in the end stage
Sx are often missed until late but reduce QOL

Question 2

Aetiology and risk factors for primary billiary cirrhosis

Answer 2

PBC is believed to have autoimmune component (ANA)
But main way-initial insult leads to fibrosis and secondary insults but the bile salts to the point of cirrhosis. Cholestasis-> pooling of bile-> 2nd are damage to liver and liver failure

risk factors
Women (10x)
Age (55-65)
FHx of PBC or AID
Smoking
UTI
Hx of hypercholestermia

Question 3

Epidiemology of primary billiary cirrhosis

Answer 3

35 per 100000 so relatively commoner than other
Much more common in men, elderly
might be 1 in 750 in elderly white women

Question 4

Signs and Sx of primary billiary cirrhosis

Answer 4

Most common:
Pruritus
Fatigue

Happens: sleep distrubance
hepatomegaly
Postural diziness

expect jaundice but not actually always there

as liver fails-ascites, portal hypertense etc

Question 5

Investigations of primary billiary cirrhosis

Answer 5

Bloods
LFTs-obstructive jaundice picture-high ALP, elevated GGT, norm/high ALT
Billirubin elevated
Albumin drops as liver damage

ANA Abs’
Immunofluresence or elisa

abdo USS-make sure nothing is obstructive