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Primary Sclerosing Cholangitis (Cancer Risk (bile duct cancer, gall…
Primary Sclerosing Cholangitis
Pathology
Progressive cholestasis with bile duct inflammation and strictures
Presentation
Pruritus
Fatigue
Advanced
Ascending cholangitis
Cirrhosis
End stage hepatic failure
Associations
female
autoimmune hepatitis
IBD - more in UC
colorectal cancer
Cancer Risk
bile duct cancer
gall bladder cancer
colorectal cancer
liver cancers
so do yearly colonoscopy and USS
consider cholecystectomy for gallbladder polyps
Ix
:arrow_up:ALP
:arrow_up:bilirubin
hypergammaglobinaemia
AMA -ve
ANA :heavy_plus_sign:maybe
SMA :heavy_plus_sign:maybe
ANCA :heavy_plus_sign: maybe
ERCP
distinguishes large duct from small duct disease
Liver biopsy
fibrous obliterative cholangitis
Mx
Liver transplant
For end stage disease
reoccurence in up to 30%
those with IBD have 5-10% chance developing colorectal cancer post transplant
Ursodeoxycholic acid
may protect against colorectal cancer
:check:LFTs
Cholestyramine
for prutitus
Abx for bacterial cholangitis