Primary Sclerosing Cholangitis (PSC) Flashcards
What is primary sclerosing cholangitis (PSC)?
A biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts
Common in men and associated with ulcerative colitis (UC)
WHO is PSC mostly commonly associated with?
- Men
- PMHx: UC
What percentage of patients with ulcerative colitis have PSC?
4%
80% of patients with PSC have ulcerative colitis
What are common associations with primary sclerosing cholangitis?
- Ulcerative colitis
- Crohn’s disease (much less common association)
- HIV
What is cholestasis?
A condition where the flow of bile from the liver to the duodenum is impaired
List common features of primary sclerosing cholangitis.
- Cholestasis
- RUQ pain
- Jaundice
- Pruritus
- Raised bilirubin
- Raised ALP
- Fatigue
What are the standard diagnostic investigations for PSC?
- Endoscopic retrograde cholangiopancreatography (ERCP)
- Magnetic resonance cholangiopancreatography (MRCP)
What does ERCP or MRCP show in cases of PSC?
Multiple biliary strictures giving a ‘beaded’ appearance
What is the significance of p-ANCA auto-antibodies in PSC?
It may be positive in patients with PSC
What might liver biopsy show in PSC?
Fibrous, obliterative cholangitis often described as ‘onion skin’
What is the first line management for PSC?
- ** Ursodeoxycholic acid**
- Supportive care (fat-soluble vitamin supplementation, regular screening for cholangiocarcinoma, Vit D)
- ** Cholestyramine **for pruritus
What are the surgical options for managing PSC?
- ERCP with stenting
- Liver transplant (complete cure!)
What are potential complications of primary sclerosing cholangitis?
- Cholangiocarcinoma (in 10%)
- Increased risk of colorectal cancer
