Primary Biliary Cholangitis

Question 1

What is the pathophysiology of primary biliary cholangitis?

Answer 1

Bile ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis which leads to fibrosis, cirrhosis, and portal hypotension.

Question 2

What is the hallmark antibody in primary biliary cholangitis?

Answer 2

Antimitochondrial antibodies

Question 3

How is suspected primary biliary cholangitis investigated?

Answer 3

1. Incidental raised ALP
2. Lethargy, pruritus preceding jaundice.
3. Cholestatic picture of LFTs, USS to exclude extrahepatic causes of cholestasis.

Question 4

What is the treatment for primary biliary cholangitis?

Answer 4

Treat pruritus, fat soluble vitamins, 2x yearly USS and AFP, transplant for end stage.