PSC Flashcards
What is PSC?
chronic cholestatic liver disease with progressive inflammatory fibrosis + obliteration of intrahepatic + extrahepatic bile ducts
Describe the aetiology of PSC
UNKNOWN
Possible immune + genetic predisposition with environmental triggers
Close association with IBD (esp. UC)
Explain the relationship between PSC and UC
4/5 of PSC pts have UC
Most UC pts DONT have PSC
Describe the epidemiology of PSC
25-40y
M > F
List 5 symptoms of PSC
Pruritis RUQ pain Weight loss Fatigue Fever + rigors caused by acute cholangitis (less common)
List 5 signs of PSC
Intermittent Jaundice Hepatosplenomegaly Spider naevi Palmar erythema Ascites
What is the pathogenesis of PSC?
Periductal inflammation with periductal concentric fibrosis Portal oedema Bile duct proliferation Expansion of portal tracts Progressive fibrosis Development of biliary cirrhosis
What should you look for in the PMH if PSC is suspected?
UC
How may PSC be missed?
If Asymptomatic
May be diagnosed after persistently raised ALP
What is seen on LFTs in PSC?
High ALP + GGT
Mildly elevated ALT + AST
Low albumin + high BR (later stages)
Describe the serology found in PSC
IgG high in children IgM high in adults pANCA present in 70% ASMA + ANA present in 30% Anti-mitochondrial antibodies (AMA) usually ABSENT
What is seen on ERCP in PSC?
Stricturing + interspersed dilation of intrahepatic + (occasionally) extraheptic bile ducts
Small diverticuli on the CBD may be seen
Why perform MRCP in PSC?
Enables non-invasive imaging of the biliary tree
Wh perform liver biopsy in PSC?
Confirms dx
Allows staging
Which ducts are affected in PSC?
INTRAHEPATIC
EXTRAHEPATIC
