Primary Biliary Cirrhosis Flashcards
1
Q
What is primary biliary cirrhosis?
A
Immune-mediated chronic progressive cholestatic liver disease with destruction of intrahepatic bile ducts
2
Q
Major histocompatability complex gene family associted with primary biliary cirrhosis?
A
DRB1*008
3
Q
What is found in over 95% of those with PBC?
A
Circulating antimitochondrial Abs (AMAs)
4
Q
What is the unique lesion found ing stage I PBC duct lesion?
A
Chronic Destructive Cholangitis affecting small and medium-sized intrahepatic bile ducts
5
Q
What occurs in stage 2 of PBC?
A
Scarring
6
Q
What occurs in stag 3 of PBC?
A
Cirrhosis - Dark green bile-stained liver that exhibits fine nodularity
7
Q
In what gender is 90-95% affected by PBC?
A
Women
8
Q
Clinical presentation of PBC?
A
- Initially - Severe pruritus
- Xanthomas, steatorrhea
- Osteomalacia/porosis due to vitamin D malabsorption
- Cirrhosis
