Primary Biliary Cirrhosis

Question 1

What is primary biliary cirrhosis?

Answer 1

AI condition associated with granuloma formation in the bile ducts

Question 2

What is the patho of primary biliary cirrhosis?

Answer 2

inflammatory disorder causing destruction of interlobular bile ducts

Question 3

What are RF for primary biliary cirrhosis?

Answer 3

1. Middle aged female

2. PMHx of other AI conditions

Question 4

What may primary biliary cirrhosis go to?

Answer 4

later stages may progress to liver failure

Question 5

What are signs and symptoms of primary biliary cirrhosis?

Answer 5

1. Pruritus
2. Cholestatic jaundice
3. RUQ pain
4. Xanthelasma and xanthomata
5. Clubbing
6. Hepatosplenomegaly
7. Fatigue

Question 6

What is cholestatic jaundice?

Answer 6

due to retention of bile salts causing fat malabsorption features and itching

Question 7

What Ix are done for primary biliary cirrhosis?

Answer 7

bloods

Question 8

What blood tests are done for primary biliary cirrhosis?

Answer 8

1. AMA M2 antibodies - diagnostic
2. SMA
3. raised serum IgM

Question 9

What is the management of primary biliary cirrhosis?

Answer 9

1. Cholestyramine for pruritus
2. Vitamin A,D,E,K supplements
3. Ursodeoxycholic acid
4. Liver transplant if BR >100

Question 10

What doe cholestryramine do?

Answer 10

reduces the build up of bile salts which drives the itching

Question 11

What does Ursodeoxycholic acid do?

Answer 11

promotes bile flow and is hepatoprotective

Question 12

What is the cause of PBC?

Answer 12

granulomatous inflammation

Question 13

What is the epid for PBC?

Answer 13

1. F>M

2. Middle aged

Question 14

What is the diagnostic feature for PBC?

Answer 14

AMA antibodies

Question 15

What are possible complication?

Answer 15

cirrhosis

Question 16

What are some associations with PBC?

Answer 16

other AI disorders