PSC Flashcards
What is PSC?
progressive liver disease with inflammatory fibrosis + obliteration of intrahepatic + extrahepatic bile ducts
Describe the aetiology of PSC
UNKNOWN
Possible immune + genetic predisposition with environmental triggers
List 3 conditions associated with PSC
UC
Crohns (much less than UC)
HIV
Explain the relationship between PSC and UC
80% of PSC pts have UC
4% UC pts have PSC
Describe the epidemiology of PSC
M > F (2:1)
25-40y
List 5 S/S of PSC
Pruritis
Jaundice
Pale stool, dark urine
RUQ pain
Fatigue
List 3 late signs of PSC
Hepatosplenomegaly
Portal hypertension
Liver failure
What should you look for in the PMH if PSC is suspected?
UC
Bloods for PSC?
FBC
LFTs, Albumin
Lipid profile
Autoantibodies
Immunoglobulins
What is seen on LFTs in PSC?
High ALP
High GGT
Norm/ high BR
Mild transaminitis/ norm ALT + AST
Describe the serology found in PSC
pANCA (80%)
ANA (75%)
IgG high
Why is imaging performed in PSC?
r/o biliary obstruction
confirm dx with MRCP
Which is the confirmatory investigation of choice in PSC?
MRCP (if no biliary obstruction)
(ERCP if obstruction)
What is seen on MRCP in PSC?
multifocal intrahepatic + extrahepatic bile duct strictures
Why perform liver biopsy in PSC?
Not routinely performed in workup
Performed in small duct/ AI hepatitis overlap syndrome
What may be seen on biopsy in PSC?
concentric periductal “onion skin” fibrosis
Which ducts are affected in PSC?
INTRAHEPATIC
EXTRAHEPATIC
Describe management of PSC
Mainly supportive
No medications prevent progression
Describe pharmacological management of PSC
Ursodeoxycholic acid
What interventional management may be performed in PSC? What are the indications for this?
ERCP dilation +/- stenting
To relieve Sx caused by stenosis, e.g., pruritus +/or cholangitis
To exclude cholangiocarcinoma
List 4 complications of PSC
Steatorrhea + fat soluble vitamin deficiency
Cirrhosis
Bacterial cholangitis
Malignancy
Which malignancies may arise in PSC?
Cholangiocarcinoma
Gallbladder carcinoma
Colorectal cancer
Screen annually with abdo US/ MRI + colonoscopy
What is the curative treatment for PSC?
Liver transplant, but high rate recurrence