Primary Biliary Cirrhosis

Question 1

def

Answer 1

chronic autoimmune inflammatory liver disease involving progressive destruction of intrahepatic bile ducts
this causes cholestasis which leads to cirrhosis

Question 2

what can cirrhosis cause

Answer 2

portal hypertension

Question 3

aetiology

Answer 3

combination of environmental + genetic factors
unknown environmental trigger causes bile duct epithelial injury, in genetically susceptible individuals this leads to a T-cell mediated autoimmune response against bile duct epithelial cells

Question 4

what specifically are the T-cells targeting in the autoimmune response

Answer 4

self-mitochondrial proteins

Question 5

what is the hallmark of PBC

Answer 5

antimitochondrial antibodies

Question 6

epi

Answer 6

middle-age

females>males

Question 7

risk factors

Answer 7

1 family history of PBC
2 other autoimmune diseases
3 recurrent UTIs

Question 8

history

Answer 8

often asymptomatic, an incidental finding (high alkphos)

insidious onset of fatigue + pruritis

Question 9

what condition is associated with PBC

Answer 9

sjogrens syndrome

-dry eyes and mouth

Question 10

examination

Answer 10

1 early - often no signs
2 late 
-jaundice
-skin pigmentation
-xanthalasma/xanthamata
-hepatosplenomegaly

Question 11

what are the signs of chronic liver disease

Answer 11

1 clubbing
2 palmar erythema
3 spider naevi

Question 12

investigations

Answer 12

1 bloods
-LFTs (high alkphos, high GGT)
-AMA
2 USS
-exclude extrahepatic cholestasis (biliary obstruction due to gallstone)
3 liver biopsy

Question 13

what blood results would be found in late disease

Answer 13

high bilirubin (jaundice)
prolonged PT