Primary Sclerosing Cholangitis

Question 1

what is PSC

Answer 1

intrahepatic and extra hepatic ducts become strictured and fibrotic with inflammation
obstruction to bile

this can cause back pressure on the liver which leads to inflammation, fibrosis and cirrhosis

Question 2

which disease is associated with PSC

Answer 2

UC

Question 3

risk factors for PSC

Answer 3

male
30s-40s
UC
FHx

Question 4

presentation of PSC

Answer 4

jaundice
chronic RUQ pain
puritis
fatigue
hepatomegaly
signs of cirrhosis

Question 5

what might you find on LFTs suggesting PSC

Answer 5

cholestatic picture

significantly increased ALP
increased billirubin

in later disease ALT and AST may increase as the disease progressess to hepatitis

Question 6

which antibodies are linked to PSC

Answer 6

ANA
p ANCA
anti cardioipid (aCL)

Question 7

what is the gold standard investigation for PSC

Answer 7

MRCP
shows bile duct leisions or strictures

Question 8

what are complications of PSC

Answer 8

acute bacterial cholangitis
cholangiocarcinoma
colorectal cancer
liver cirrhosis and failure
deficiencies in fat soluble vitamins

Question 9

medical managment options for PSC

Answer 9

ursodeoxycholic - slows progression
cholestyramine - bile acid sequestrant

Question 10

surgical options for management of PSC

Answer 10

ERCP for stenting to relieve symptoms
only curative option is liver transplant