Liver disease of less common aetiology - primary sclerosing cholangitis (PSC)
Description
- Chronic fibro-inflammatory disease of intra- and/or extrahepatic bile ducts -> multifocal strictures + intervening dilatation -> biliary cirrhosis
- Cholestatic biochemistry: inc ALP and GGT, with fluctuating bilirubin
Subtypes
| Large-duct (classic) ~90% | Intra- and/or extrahepatic strictures on MRCP |
| Small-duct ~10% | Normal cholangiogram, PSC on biopsy. Better prognosis; low cholangiocarcinoma risk |
| PSC-AIH overlap | More common in children/young adults; steroid-responsive |
| IgG4-related sclerosing cholangitis | A mimic, not a subtype - steroid-responsive, must not be missed |
- Progressive and, unlike PBC, has no effective medical therapy
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