Primary Biliary Cholangitis

Question 1

What is primary biliary cholangitis?

Answer 1

Autoimmune condition which is liver specific, anti-mitochondrial M2 antibodies target the E2 and E3 subunits in the inner leaflet of mitochondrial membrane.

Question 2

What is PBC thought to be mediated by?

Answer 2

CD4+ Mediated

Question 3

Who gets PBC?

Answer 3

Middle aged
Morecommon in men
Can be caused by gallstones though which are more common in women (4F’s)

Question 4

Presentation of PBC?

Answer 4

Normally asymptomatic
Fatigue
Jaundice
RUQ Pain
Itch without rash
Xanthelesma & xanthomas

Question 5

Diagnosis of PBC?

Answer 5

ERCP
-2/3 of these:
Positive antimicrobial antibody
Cholestatic LFTs- alk phos and gamma GT elevated more than ALT
Liver biopsy

Question 6

1st line treatment of Primary Biliary cholangitis

Answer 6

Urseo deoxycholic acid

Question 7

What does urseo deoxycholic aciddo?

Answer 7

Increases flow of bile from the liver washing toxic bile salts out

Question 8

2nd line treatment of PBC?

Answer 8

Obeticholic acid

Question 9

What does obeticholic acid do?

Answer 9

Nuclear receptor function changing inflammatory cholesterol metabolism improving inflammation in the liver

Question 10

Prognosis of PBC?

Answer 10

Most don’t get symptoms

Even those who do, don’t liver failure