Primary sclerosing cholangitis
From Ganfyd
Web Resources for Primary sclerosing cholangitis
Relevant Clinical Literature
UK Guidance
Other Wikis
Medpedia on Primary sclerosing cholangitis (Less technical, good quality control)
Wikipedia on Primary sclerosing cholangitis (Less technical, ? quality control)
Primary sclerosing cholangitis(PSC) is a cholestatic disorder of unknown aetiology, possibly autoimmune mediated. It results in patchy stricturing of the extra-hepatic bile ducts.
Association with inflammatory bowel disease (IBD). 70-80% of patients have IBD, while 2-4% of IBD patients have PSC.
Diagnosis
- Imaging of biliary tree, i.e. cholangiography via ERCP, MRCP or PTC.
- Liver enzymes consistent with cholestatic picture.
- Associated with raised serum p-ANCA
- Biopsy may be helpful but no single histological feature is pathognomonic.
- No other convincing alternative diagnoses.
Treatments
None proven to significantly improve survival.
- Ursodeoxycholic acid
- Steroids
- Stenting
- Stone extraction
- Liver transplantation
