Gastro Flashcards
Primary Sclerosing Cholangitis
what is it
the intrahepatic or extrahepatic ducts become strictured + fibrotic
Primary Sclerosing Cholangitis
pathophysiology
strictured + fibrotic intrahepatic or extrahepatic ducts
obstruction to flow of bile out liver and into intestines
chronic bile obstruction leads to hepatitis, fibrosis and cirrhosis
Primary Sclerosing Cholangitis
what does sclerosis refer to
stiffening and hardening of the bile ducts
Primary Sclerosing Cholangitis
what does cholangitis refer to
inflammation of the bile ducts
Primary Sclerosing Cholangitis
what condition is it associated with
ulcerative colitis
Primary Sclerosing Cholangitis
cause
unclear but probs a combination of:
- genetic
- autoimmune
- intestinal microbiome
- environmental factors
Primary Sclerosing Cholangitis
RFs (4)
- male
- aged 30-40
- ulcerative colitis
- FH
Primary Sclerosing Cholangitis
presentation (5)
- jaundice
- chronic RUQ pain
- pruritis
- fatigue
- hepatomegaly
Primary Sclerosing Cholangitis
what will LFTs show
a ‘cholestatic’ picture
ALP is the most deranged
Primary Sclerosing Cholangitis
LFTs: why may there be a rise in bilirubin
as the strictures become more severe and prevents bilirubin from being excreted through the bile duct
Primary Sclerosing Cholangitis
LFTs: why may other LFTs apart from ALP be deranged
as the disease progresses to hepatitis
Primary Sclerosing Cholangitis
are there any antibodies which are highly sensitive or specific to PSC
no but can indicate where there is an autoimmune element that may respond to immunosuppression
Primary Sclerosing Cholangitis
which autoantibodies may be present
p-ANCA (94%)
ANA (77%)
aCL (63%) - anticardiolipid
Primary Sclerosing Cholangitis
gold standard inx for dx
MRCP - magnetic resonance cholangiopancreatography (MRI scan of the liver, bile ducts and pancreas)
Primary Sclerosing Cholangitis
what will the MRCP show
may show bile duct lesions or strictures
Primary Sclerosing Cholangitis
associations and complications
- acute bacterial cholangitis
- cholangiocarcinoma
- colorectal cancer
- cirrhosis and liver failure
- biliary strictures
- fat soluble vitamin deficiencies
Primary Sclerosing Cholangitis
mnx
- liver transplant
- ERCP
- Colestyramine
Primary Sclerosing Cholangitis
mnx: what is ERCP
endoscopic retrograde cholangio-pancreatography
insert camera through throat through to bile ducts.
Use X-rays and injecting contrast to identify any strictures
stented
Primary Sclerosing Cholangitis
mnx: what is colestyramine
a bile acid sequestrate: binds to bile acids to prevent absorption in the gut
can help with pruritus due to raised bile acids
Primary Biliary Cirrhosis
what is it
the immune system attacks the small bile ducts within the liver
Primary Biliary Cirrhosis
how does it lead to fibrosis, cirrhosis and liver failure
immune system attacks the intralobar ducts (aka Canals of Hering)
this causes obstruction of the outflow of bile (cholestasis)
back pressure of bile obstruction leads to fibrosis
Primary Biliary Cirrhosis
what causes itching
obstruction to the outflow of bile acids so they build up in the blood as they are not being excreted
Primary Biliary Cirrhosis
what causes jaundice
obstruction to the outflow of biliruibin so they build up in the blood as they are not being excreted
Primary Biliary Cirrhosis
what causes raised cholesterol
obstruction to the outflow of cholesterol so they build up in the blood as they are not being excreted