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Primary sclerosing cholangitis

Last updated: August 5, 2021

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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.

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

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

References:[2]

Laboratory findings

Imaging

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 of cholestatic biliary disease

Primary sclerosing cholangitis Primary biliary cholangitis

Secondary sclerosing cholangitis [3][4]

Epidemiology
  • More common among middle-aged men

  • More common among middle-aged women

  • Depends on the underlying condition
Pathophysiology
  • 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.

References:[6][7][8][9]

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

  1. Abdalian R, Heathcote EJ. Sclerosing cholangitis: A focus on secondary causes. Hepatology. 2006; 44 (5): p.1063-1074. doi: 10.1002/hep.21405 . | Open in Read by QxMD
  2. Ruemmele P, Hofstaedter F, Gelbmann CM. Secondary sclerosing cholangitis. Nature Reviews Gastroenterology & Hepatology. 2009; 6 (5): p.287-295. doi: 10.1038/nrgastro.2009.46 . | Open in Read by QxMD
  3. 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.
  4. 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.
  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): p.187-198. doi: 10.1016/j.bpg.2017.04.004 . | Open in Read by QxMD
  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): p.455-9.
  8. Poropat G, Giljaca V, Stimac D, Gluud C. Bile acids for primary sclerosing cholangitis. The Cochrane Database of Systematic Reviews . 2011 : p.CD003626.
  9. Kremer AE, Namer B, Bolier R et al. Pathogenesis and Management of Pruritus in PBC and PSC. Digestive Diseases. 2015; 33 (Suppl 2): p.164-75. doi: 10.1159/000440829 . | Open in Read by QxMD
  10. Gochanour E,Jayasekera C, Kowdley K. Primary Sclerosing Cholangitis: Epidemiology, Genetics, Diagnosis, and Current Management. Clinical Liver Disease. 2020; VOL 15 (NO 3).
  11. Diagnosis and Management of Primary Sclerosing Cholangitis. https://www.aasld.org/sites/default/files/2019-06/PrimarySclerosingCholangitis2010.pdf. Updated: August 31, 2009. Accessed: January 13, 2021.
  12. Ponsioen C. Diagnosis, prognosis, and management of primary sclerosing cholangitis. Gastroenterology & Hepatology. 2013; 9 (7): p.453-65.