PSC and PBC

Question 1

Primary biliary cholangitis

• gender most affected
• immune mechanism
• serological markers
• clinical signs

Answer 1

females most affected
T-cell mediated
AMA + in 95% and ANA + 70%, HDL and ALP “strikingly elevated”
Fatigue, pruritis, xanthomas, jaundice, dematographism

Question 2

PBC associations

Answer 2

Sjogrens (40-65%),
Hashimotos thyroiditis,
Lc-Systemic sclerosis (anticentromere Abs) (5-15%),
RA (5-10%).

Question 3

PBC complications

Answer 3

Cirrhosis
HCC
Metabolic bone disease
Malabsorption and steatorrhoea

Question 4

Diagnostic approach for PBC

Answer 4

1. No extrahepatic biliary obstruction
2. 2 of the following 3
   - ALP 1.5X ULN
   - AMA titre >1:40
   - histological evidence of PBC

Question 5

Treatment for PBC

Answer 5

Ursodeoxycholic acid
- improves transplant free survival
Transplant

Question 6

Primary sclerosing cholangitis

• gender breakdown
• percent who also have UC
• percent of those with UC who have PSC

Answer 6

M>F
up to 90% have UC
<10% with UC have PSC

Question 7

Primary sclerosing cholangitis

• serological markers
• symptoms
• distribution of disease
• imaging findings

Answer 7

pANCA 80%, ANA 75%, elevated IgM 50%
Jaundice, hepatomegaly, fatigue, pruritis (latter can be associated with normal bilirubin)
Intra and extrahepatic in 87%, intrahepatic only in 11% and extrahepatic only in 2%
“beaded” appearance of ducts, areas of stricturing and dilatation

Question 8

PSC complications

Answer 8

• Fat-soluble vitamin deficiencies (ADEK)
  
  • Metabolic bone disease
  • Cholangitis and lithiasis
  • Cholangiocarcinoma
  • HCC (in those with cirrhosis)
    
    • Colon cancer (in those with UC too)

Question 9

Treatment for PSC

Answer 9

Transplant