Primary Sclerosing Cholangitis (PSC) Flashcards
Define PSC
chronic cholestatic liver disease characterized by progressive inflammatory fibrosis, obliteration and stricturing of intrahepatic and extrahepatic bile ducts
RFs of PSC
Male
Age 30-40
Ulcerative colitis
FH
Aetiology of PSC
Unsure but likely to be multifactorial between genetic, autoimmune, and microbiome
70% of PSC is associated with established ulcerative colitis
Epidemiology of PSC
30-40 age
70% are men
2-7 per 100,000
Presenting symptoms of PSC
Jaundice
RUQ pain (chronic)
Pruritis (itching)
Fatigue, fever, wt loss
Signs of PSC
Hepatomegaly Jaundice Spider naevi Palmar erythema Ascites WT loss
Investigations for PSC and state what findings would be indicative for PSC
Bloods LFTs = alkaline phosphatase increased the most, bilirubin increased, AST/ALT will also increase in progressive disease
Serology - autoantibodies can show whether there’s an autoimmune element to the disease. P-ANCA (anti-neutrophil cytoplasmic AB), ANA (anti-nuclear AB) and aCL (anti-cardiolipin AB) will be increased
How to manage PSC
Liver transplant
ERCP - puts stents into strictures via endoscope
Ursodeoxycholic acid - slows progression
Cholestyramine - bile sequestrate (prevents bile absorption, helps itching)
Complications of PSC
Cirrhosis, liver failure
Cholangiocarcinoma (must monitor them for this)
Acute bacterial cholangitis
Deficiency in fat soluble vitamins
Describe what happens in PSC
Intra and extrahepatic bile ducts become strictured and fibrotic and have inflammation. Narrowing and hardening of the bile ducts causes an obstruction, causing cholestasis. Over time the backpressure of the bile into the liver causes
How to diagnose a PSC?
MRCP - MRI of liver, bile ducts, pancreas
Presence of bile duct lesions/strictures
