What is Primary Sclerosing cholangitis (PSC )?

Primary Sclerosing cholangitis (PSC )

  • Immunological destruction of the intrahepatic and extrahepatic bile ducts is the main pathological feature
  • It is currently believed that PSC is an immunologically mediated disease, triggered in genetically susceptible individuals by toxic or infectious agents, which may gain access to the biliary tract through a leaky, diseased colon
  • 90% of PSC is associated with inflammatory bowel disease, particularly ulcerative colitis, Ulcerative colitis (UC) is the most frequent association with primary sclerosing cholangitis. 
  • Around 70% of the patients diagnosed with PSC are men. The time from the diagnosis to the end-stage hepatic failure is thought to be around 12 years 

Features of PSC: 

  • Features of cholestasis: jaundice, pruritus 
  • Fatigue 
  • Right upper quadrant pain
  • Intermittent diarrhoea (if associated with UC)

Investigations of PSC:

  • ERCP: the gold standard Diagnostic test which shows multiple intrahepatic and extrahepatic bile duct strictures and dilatations, giving a 'beaded' appearance. It is a therapeutic tool as well
  • MRCP: The non-invasive MRCP is often performed initially. MRCP would be the initial diagnostic investigation of choice, particularly given a lower complication rate and its ability to image ducts proximal to obstructing strictures
  • Liver biopsy: Onionskin scarring (arrows) surrounding a bile duct
  • Increase in IgM in 84 % of cases
  • P-ANCA may be positive in 65-85% of cases
  • Positive ANA in 55% of cases
Primary Sclerosing cholangitis MRCP

[The gold standard diagnostic tool is ERCP ||| The initial diagnostic investigation of choice is MRCP]

Liver biopsy in primary Sclerosing cholangitis

Complications of PSC:

  • Complications due to chronic cholestasis, notably steatorrhoea, fat-soluble vitamin malabsorption, large biliary strictures, cholangitis
  • Cholangiocarcinoma, and 
  • Colonic carcinoma

Treatments of PSC: (There are no medications that greatly retard the progression of PSC)

  • The definitive treatment for PSC is hepatic transplantation 
  • Mechanical relief can be obtained by placement of a stent or by balloon dilatation performed at ERCP
  • Cholestyramine is given along with ursodeoxycholic acid (UDCA) to reduce pruritus
  • Fat-soluble vitamin supplementation is necessary owing to steatorrhoea
  • Antibiotic prophylaxis during instrumentation of the biliary tree is mandatory to reduce the risk of bacterial cholangitis and also used in an acute attack of cholangitis without instrumentation (Ciprofloxacin is the prophylactic antibiotic drug of choice before ERCP)

[ images are reproduced from Davidson's Principles and Practice of Medicine; 23rd Edition, Fig 22.33 and 22.34]
[Information from:
  • Rapid review of clinical medicine for MRCP part 2, 2nd Edition, by Sanjay Sharma and Rashmi Kaushal; question no: 6
  • Davidson's Principles and Practice of Medicine; 23rd Edition; page: 889
  • Step Up To MRCP Review Note For Part I & Part II By Dr Khaled El Magraby; page 279]

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