DR Medhat MRCP

Primary sclerosing cholangitis (PSC)
- Is a biliary disease of unknown aetiology characterised by inflammation and fibrosis
of intra and extra-hepatic bile ducts.
• Features :
Cholestasis.
• Associations :
Ulcerative colitis : 4% of patients with UC have PSC, 80% of patients with PSC
have UC
Crohn's (much less common association than UC).
HIV.
• Investigation :
1) ERCP :
- Is the standard diagnostic tool .
- Showing multiple biliary strictures giving a 'beaded' appearance
2) ANCA may be positive.
3) liver biopsy :
- Of limited role
- May show fibrous, obliterative cholangitis often described as ‘onion skin’
• Complications
Cholangiocarcinoma (in 10%)
Increased risk of colorectal cancer.
• Treatment : same as PBC (cholestyramine , ursodeoxycholic acid ,liver transplantation)
ERCP shows multiple strictures & dilatations
(beaded appearance)
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