Primary Sclerosing Cholangitis Flashcards
What is Primary Sclerosing Cholangitis?
- Intra- and extra-hepatic ducts become strictured and fibrotic
- Bile outflow obstruction due to the sclerosis (stiffening) and cholangitis (inflammation)
What causes Primary Sclerosing Cholangitis?
Unclear, but likely a mix of genetics, autoimmunity and environmental factors
What is Primary Sclerosing Cholangitis associated with?
UC
What are the non-modifiable risk factors of Primary Sclerosing Cholangitis?
- Age 30-40
- Male
- UC
- Family history
How does Primary Sclerosing Cholangitis present?
- Jaundice
- Chronic RUQ pain
- Pruritis
- Fatigue
- Hepatomegaly
What do LFTs show in PSC?
Cholestasis (high ALP, rest will rise later in the disease course)
Why may autoantibodies be useful for PSC?
Not very helpful in diagnosis but may identify an AI component that may be targeted with immunosuppression
What is the gold standard diagnostic for PSC?
MRCP (Non-invasive)
- MRI of the liver, bile ducts and pancreas
- Shows strictures and lesions
What are the complications of PSC?
- Acute bacterial cholangitis
- Cholangiocarcinoma
- Colorectal Ca
- Cirrhosis
- Vit ADEK deficiency
How is PSC managed?
- ERCP and stenting
- Ursodeoxycholic acid
- Cholestyramine
What is ERCP?
- Endoscope to the sphincter of Oddi and ampulla of Vater
- Use XR and contrast to see and stent strictures
How does ursodeoxycholic acid work?
Reduces intestinal cholesterol absorption
How does cholestyramine work?
Bile acid sequestrant
