• Clinical science

Primary sclerosing cholangitis


Primary sclerosing cholangitis (PSC) is a progressive chronic inflammation of both the intrahepatic and extrahepatic bile ducts. While the exact etiology is unknown, there is a strong association with autoimmune diseases, particularly ulcerative colitis (UC). In the early stages, PSC is usually asymptomatic. Later in the course of the disease, patients present with symptoms of cholestasis (e.g., pruritus, jaundice). Laboratory abnormalities include elevated liver function tests and autoantibodies (pANCA in up to 80% of cases). Magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP) is performed to confirm the diagnosis. Management is primarily symptomatic, with liver transplantation reserved for end-stage liver disease.


  • Sex: > (2:1)
  • Age: The median age at diagnosis is ∼ 40.


Epidemiological data refers to the US, unless otherwise specified.


The majority of PSC patients also have ulcerative colitis.References:[2][1]

Clinical features



Laboratory findings


  • Cholangiography
    • Method of choice: magnetic resonance cholangiopancreatography (MRCP)
    • Alternatives
      • Endoscopic retrograde cholangiopancreatography (ERCP)
        • More invasive but also more accurate than MRCP
        • Good alternative for patients who cannot undergo MRI testing (e.g., patients with pacemaker)
      • Percutaneous transhepatic cholangiography (PTC)
        • Most invasive test
        • For patients who cannot undergo ERCP
    • Findings: multifocal strictures alternating with dilation and beading of bile ducts
  • Ultrasound
    • Irregular diameter of the bile duct
    • Diffuse thickening of the wall of the common hepatic and bile ducts
  • Other: colonoscopy (to assess for UC)

Liver biopsy

  • Not an essential part of the workup; usually done if small duct PSC is suspected, which is not always detectable via cholangiography
  • Typical finding: concentric "onion skin" scarring and fibrosis of bile ducts

If a patient with pre-existing chronic inflammatory bowel disease displays increased ALP, GGT, and conjugated bilirubin, always consider PSC.References:[1][2]

Differential diagnoses

Differential diagnoses of cholestatic biliary disease

Primary sclerosing cholangitis Primary biliary cholangitis

Secondary sclerosing cholangitis [3][4]

  • More common among middle-aged men

  • More common among middle-aged women

  • Depends on the underlying condition
  • Progressive chronic inflammation of both intrahepatic and extrahepatic bile ducts

  • Progressive destruction of only intrahepatic small and medium-sized bile ducts

Clinical presentation
  • Similar to PSC
  • Additional symptoms corresponding to the underlying condition
  • Poor prognosis
Laboratory tests
Associated conditions
  • Autoimmune conditions

The differential diagnoses listed here are not exhaustive.





We list the most important complications. The selection is not exhaustive.

  • 1. Kowdley KV. Primary sclerosing cholangitis: Epidemiology and pathogenesis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/primary-sclerosing-cholangitis-epidemiology-and-pathogenesis. Last updated January 13, 2015. Accessed December 26, 2016.
  • 2. Kowdley KV. Primary sclerosing cholangitis in adults: Clinical manifestations and diagnosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/primary-sclerosing-cholangitis-in-adults-clinical-manifestations-and-diagnosis. Last updated January 4, 2016. Accessed December 26, 2016.
  • 3. Abdalian R, Heathcote EJ. Sclerosing cholangitis: A focus on secondary causes. Hepatology. 2006; 44(5): pp. 1063–1074. doi: 10.1002/hep.21405.
  • 4. Ruemmele P, Hofstaedter F, Gelbmann CM. Secondary sclerosing cholangitis. Nature Reviews Gastroenterology & Hepatology. 2009; 6(5): pp. 287–295. doi: 10.1038/nrgastro.2009.46.
  • 5. Visseren T, Darwish Murad S. Recurrence of primary sclerosing cholangitis, primary biliary cholangitis and auto-immune hepatitis after liver transplantation. Best Practice & Research Clinical Gastroenterology. 2017; 31(2): pp. 187–198. doi: 10.1016/j.bpg.2017.04.004.
  • 6. Kowdley KV. Primary sclerosing cholangitis in adults: Management. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/primary-sclerosing-cholangitis-in-adults-management. Last updated January 12, 2016. Accessed December 26, 2016.
  • 7. Van thiel DH, Carroll P, Abu-elmagd K, et al. Tacrolimus (FK 506), a treatment for primary sclerosing cholangitis: results of an open-label preliminary trial. The American Journal of Gastroenterology. 1995; 90(3): pp. 455–9. pmid: 7532912.
  • 8. Poropat G, Giljaca V, Stimac D, Gluud C. Bile acids for primary sclerosing cholangitis. The Cochrane Database of Systematic Reviews . 2011: p. CD003626. pmid: 21249655.
  • 9. Kremer AE, Namer B, Bolier R et al. Pathogenesis and Management of Pruritus in PBC and PSC. Digestive Diseases. 2015; 33(Suppl 2): pp. 164–75. doi: 10.1159/000440829.
  • Ponsioen C. Diagnosis, prognosis, and management of primary sclerosing cholangitis. Gastroenterology & Hepatology. 2013; 9(7): pp. 453–65. url: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3736784/.
last updated 11/13/2020
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