Primary biliary cirrhosis

Question 1

Define primary biliary cirrhosis

Answer 1

Chronic inflammatory liver disease involving progressive destruction of intrahepatic bile ducts, leading to cholestasis & ultimately cirrhosis

Question 2

Aetiology of primary biliary cirrhosis

4

Answer 2

UNKNOWN
Likely to be autoimmune
Genetic & environmental factors involved
An environmental trigger may cause bile duct epithelial injury, which leads to T cell mediated autoimmune response directed against bile duct epithelial cells

Question 3

Epidemiology of primary biliary cirrhosis

group

Answer 3

Mainly affects middle aged women (9:1 female male)

Question 4

Presenting symptoms of primary biliary cirrhosis

5

Answer 4

May be an incidental finding on blood tests
Insidious onset w/ vague symptoms such as:
fatigue
weight loss
pruritus
Rarely can cause discomfort in RUQ
May present w/ complications of liver decompensation (e.g. jaundice, ascites, variceal haemorrhage)
May present w/ symptoms for associated conditions (e.g. Sjogren’s syndrome, arthritis, Raynaud’s phenomenon)

Question 5

Signs of primary biliary cirrhosis on physical examination - early

Answer 5

May be no signs

Question 6

Signs of primary biliary cirrhosis on physical examination - late
(7)

Answer 6

Jaundice 
Skin pigmentation 
Scratch marks
Xanthomas (2º to hypercholesterolaemia)
Hepatomegaly
Ascites
Signs of chronic liver disease

Question 7

Investigations for primary biliary cirrhosis

3

Answer 7

Bloods
US
Liver biopsy

Question 8

Investigations for primary biliary cirrhosis - bloods

6

Answer 8

LFTs
high ALP & GGT
bilirubin may be high or normal
ALT & AST normal initially but increase as disease progresses & cirrhosis develops

Clotting
prolongation of PT

Anti mitochondrial antibodies (typical feature)

High IgM

High cholesterol

TFTs
PBC associated w/ autoimmune thyroid disease

Question 9

Investigations for primary biliary cirrhosis - US

Answer 9

To exclude extra hepatic biliary obstruction (e.g. gallstones)

Question 10

Investigations for primary biliary cirrhosis - liver biopsy

Answer 10

Shows chronic inflammatory cells & granulomas around the intrahepatic bile ducts, destruction of bile ducts, fibrosis & regenerating nodules of hepatocytes