Fact-sheet: Primary sclerosing cholangitis
Updated on 09/17/2021 at 12:16 PM
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Definition
• Disease of young patients, predominantly affecting men (2/3 of cases)
• Frequent association with IBD (mainly ulcerative colitis)
• Exact cause unknown (autoimmune?)
• Diagnosis relies on the combination of 4 types of features:
- Biological: anicteric cholestasis
- Radiological: morphological abnormalities of the intra- and extrahepatic bile ducts
- Histological: fibrous or obliterative cholangitis
- Association with another disease, particularly IBD
• Diagnosis established in the presence of 2 of these 4 criteria (including at least the histological or radiological criterion), in the absence of an identifiable etiology
MRI
• Majoie CB et al classification (AJR1991):
- Bile duct irregularities
- Beaded appearance
- Biliary rarefaction, pruned tree appearance
- Thickened bile duct walls, pseudo-diverticula
Complications:
• Cholangiocarcinoma +++
- 10-20% of cases, intrahepatic 23%, hilar 50%, common bile duct 11%
- Clinically suspected with worsening jaundice and cholestasis
- Suspicion criteria: significant irregular strictures, rapid progression of strictures, marked upstream dilation, polypoid lesions, lymphadenopathy, vascular invasion.
• Recurrence after liver transplantation.
