PBC

Question 1

What is PBC?

Answer 1

chronic inflammatory liver disease involving progressive destruction of INTRAhepatic bile ducts, leading to cholestasis, + cirrhosis

Question 2

Describe the aetiology of PBC

Answer 2

UNKNOWN
Likely AI (Genetic + environmental factors)
Environmental trigger may cause bile duct epithelial injury, which then leads to T-cell mediated AI response against bile duct epithelial cells

Question 3

Describe the epidemiology of PBC

Answer 3

F > M

Question 4

How may PBC be identified?

Answer 4

Incidental finding on blood tests
Insidious onset with vague Sx
A complication of liver decompensation
Sx of associated conditions

Question 5

List 4 symptoms of PBC

Answer 5

Fatigue
Weight loss
Pruritis
RUQ discomfort (rare)

Question 6

List 7 signs of late PBC

Answer 6

Jaundice  
Skin pigmentation  
Scratch marks  
Xanthomas (secondary to hypercholesterolaemia) 
Hepatomegaly  
Ascites  
Signs of chronic liver disease

Question 7

List 3 conditions associated with PBC

Answer 7

Sjorgens syndrome
RA
Raynaud’s phenomenon

Question 8

List 3 complications of liver decompensation that a patient with PBC may present with

Answer 8

Jaundice
Ascites
Variceal haemorrhage

Question 9

Describe LFTs you’d expect to see in PBC?

Answer 9

High ALP + GGT
BR high or normal
ALT + AST: normal initially, increase as disease progresses + cirrhosis develops
Clotting: prolongation of PT

Question 10

List 4 blood features of PBC

Answer 10

Anti-MITOCHONDRIAL antibodies
High IgM
High Cholesterol
TFTs: associated with AI thyroid disease

Question 11

Why perform an ultrasound in PBC?

Answer 11

Exclude extrahepatic biliary obstruction (e.g. gallstones)

Question 12

What is seen on liver biopsy in PBC?

Answer 12

Chronic inflammatory cells + granulomas around intrahepatic bile ducts, destruction of bile ducts, fibrosis + regenerating nodules of hepatocytes

Question 13

What ducts are affected in PBC?

Answer 13

INTRAHEPATIC