Primary Biliary Cirrhosis

Question 1

Define:

Answer 1

Chronic inflammatory liver disease involving progressive destruction of intrahepatic bile ducts leading to cholestasis and cirrhosis.

Question 2

Aetiology/risk factors:

Answer 2

UNKNOWN.

Likely to be autoimmune inflammation which leads to fibrosis, cirrhosis and portal hypertension.

Genetic and environment is involved.

Environmental trigger –> bile duct injury which T cell autoimmune response.

Question 3

Epidemiology:

Answer 3

Middle aged women (9:1 ratio female to male)

Question 4

Symptoms:

Answer 4

-Fatigue
-weight loss
-Pruritus
May have discomfort in the RUQ

Or signs of liver compensation such as ascites, jaundice and variceal haemorrhage.

May have associated conditions such as sjorgens, rheumatoid arthritis and raynauds)

Question 5

Signs:

Answer 5

Early: may have no signs

Late:

• hepatosplenomegaly
• jaundice
• skin pigementation
• stretch marks
• xanthomas
• signs of chronic liver disease

Question 6

Investigations:

Answer 6

Bloods:
LFT's = ALP and GGT are up. 
High or normal bilirubin 
ALT and AST as the disease progresses to cirrhosis
Low albumin 
High PT

AMA IS A HALLMARK SIGN OF PBC.
high IGm
high cholestrol
TFT’s- associated with autoimmune thyroid disease

US - to exclude biliary obstruction such as gallstones

Liver biopsy