Primary Sclerosing Cholangitis Flashcards
What is it?
when the intra and extra hepatic ducts become strictures and fibrotic.
Its stiffening and hardening and inflammation of the bile ducts.
the stiffening and reduced size of the lumen of the ducts causes an obstruction where the bile can’t get from the liver to the intestines.
What is does chronic obstruction of bile do?
chronic obstruction of bile causes a back pressure of bile into the liver - Liver inflammation (hepatitis) which leads to fibrosis of the liver tissue - then liver cirrhosis.
which disease is linked to primary sclerosis cholangitis
Ulcerative colitis
70% of PSC are associated with UC
Risk factors?
Male
30-40
Ulcerative colitis
presentation?
jaundice chronic RUQ pain pruritis fatigue hepatomegaly
Cirrhosis signs
Liver function tests?
Cholestatic picture (suggesting cholestasis/ slowing of bile duct flow):
Alkaline phosphatase deranged
raise in bilirubin
ALT deranged
AST deranged
Autoantibodies?
No antibodies are specific BUT they can indicate if its autoimmune and might respond to immunesuppression.
P-anca raised.
ANA raised.
Gold standard investigation?
MRCP - magnetic resonance cholangiopancreatography = MRI of liver, bile ducts and pancreas
complications?
Whenever theres blockage of the bile duct you can get acute bacterial cholangitis (infection of the bile ducts)
cholangiocarcinoma - IN 10-20%
colorectal cancer
hepatitis - cirrhosis and liver failure
fat soluble vitamin deficiency - A, D, E, K
management?
Only curative option = liver transplant
ERCP - dilate and stent strictures
bile-acid sequestrate
What causes pruritus?
increased bile acids leaking into the blood
ERCP?
endoscopic retrograde cholangiopancreatogeaphy - inset a camera through someomes throat, oesophagus stomach then the duodenum, go through the sphincter of odd, ampulla of vater then into the bile ducts.
They can dilate strictures and put a stent in.
