Primary Sclerosing Cholangitis Flashcards
Define Primary Sclerosing Cholangitis (PSC)?
A chronic cholestatic liver disease characterised by progressive inflammatory fibrosis and obliteration of intrahepatic and extrahepatic bile ducts
What is the pathology/pathogenesis of PSC?
Periductal inflammation with periductal concentric fibrosis Portal oedema Bile duct proliferation Expanse of portal tracts Progressive fibrosis Development of biliary cirrhosis
What is the aetiology of PSC?
Possible immune and genetic predisposition with environmental triggers
Close association with inflammatory bowel disease (especially UC)
UC is present in about 70% of patients with PSC
What is the epidemiology of PSC?
Usually present between 25-40 yrs of age
How is PSC diagnosed normally?
May be asymptomatic and diagnosed after persistently raised ALP
What is the normal presentation of PSC?
Intermittent jaundice Pruritis RUQ pain Weight Loss Fatigue
May present with episodes of fever and rigors caused by acute cholangitis (but this is less common)
What’s important to check for in a history of PSC?
Check for a history of UC
What are the signs of PSC on examination?
May have no signs Jaundice Hepatosplenomegaly Spider naevi Palmar erythema Ascites
What bloods do we do for PSC?
LFTs
What do we look for on LFTs for PSC?
High ALP + GGT
Mildly elevated ALT + AST
Low albumin + high bilirubin (late stages)
What Serology tests do we do for PSC?
IgG high in children
IgM high in adults
ASMA and ANA present in 30%
Anti-mitochondrial antibodies (AMA) usually absent
Perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) present in 70%
Why do we do an ERCP for PSC and what might we see?
Shows stricturing and interspersed dilation of intrahepatic and (occasionally) extrahepatic bile ducts
Small diverticuli on the common bile duct may be seen
Why do we do a MRCP for PSC?
Enables non-invasive imaging of the biliary tree
Why do we do a Liver Biopsy for PSC?
Confirm diagnosis and allows staging
