Primary Sclerosing Cholangitis Flashcards
What is PSC?
Chronic, cholestatic liver disease characterised by progressive inflammatory destruction of ANY part of the biliary tree (intra- and extra-hepatic).
Outline the epidemiology of PSC
Affects mostly young males 25-40y
Strong link with UC
o 70% patients with PSC will have UC
o 5% of patients with UC will have PSC
Outline the pathology behind PSC
Autoimmune
Periductal inflammation with concentric fibrosis gives ‘onion skin’ appearance
Inflammation associated with portal oedema and progression of fibrosis and cirrhosis
How might PSC present?
May be asymptomatic
Symptoms: intermittent jaundice, RUQ pain, pruritius, weight loss, fatigue
History of UC
Exam may show evidence of liver disease e.g. jaundice, spider naevi, palmar erythema, ascites
How would you investigate PSC?
LFTs – obstructive
Liver screen – non-specific but some say ↑ANCA
Imaging:
- USS abdo/MRCP – may show dilatation of biliary tree, if extrahepatic
- ERCP – beading of the biliary ducts (structuring and dilatation)
Biopsy – for staging of fibrosis
Endoscopy – check for UC
How would you manage PSC?
Acute cholangitis: fluid resus + antibiotics e.g. metronidazole
Symptom control:
o Colestyramine – pruritis
o Vitamin and Ca supplement
ERCP - Stenting of dominant stricture
Advanced disease – transplant
What are some complications of PSC?
↑ risk cholangiocarcinoma
Recurrent cholangitis, cirrhosis, portal HTN
