Primary Sclerosing Cholangitis
General Principles
Primary sclerosing cholangitis (PSC) is a cholestatic liver disorder characterized by inflammation, fibrosis, and obliteration of the extrahepatic and/or intrahepatic bile ducts.
- PSC can be subdivided into small duct and large duct disease. Small duct disease is defined as typical histologic features of PSC with a normal cholangiogram. In large duct disease or classic PSC, typical “beads on a string” strictures of the biliary tree can be detected by cholangiography. Small duct disease carries a more favorable prognosis.1
- The peak incidence is at about age 40 years. Most patients are middle-aged men, and the male-to-female ratio is 2:1.
- PSC is frequently associated with IBD (70% of patients have concomitant UC). The clinical course of these conditions is not correlated.
- PSC increases the risk of colon cancer, in addition to the risk conferred by IBD alone.
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Citation
Bhat, Pavat, et al., editors. "Primary Sclerosing Cholangitis." Washington Manual of Medical Therapeutics, 35th ed., Wolters Kluwer Health, 2016. The Washington Manual, www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602011/all/Primary_Sclerosing_Cholangitis.
Primary Sclerosing Cholangitis. In: Bhat PP, Dretler AA, Gdowski MM, et al, eds. Washington Manual of Medical Therapeutics. Wolters Kluwer Health; 2016. https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602011/all/Primary_Sclerosing_Cholangitis. Accessed June 4, 2023.
Primary Sclerosing Cholangitis. (2016). In Bhat, P., Dretler, A., Gdowski, M., Ramgopal, R., & Williams, D. (Eds.), Washington Manual of Medical Therapeutics (35th ed.). Wolters Kluwer Health. https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602011/all/Primary_Sclerosing_Cholangitis
Primary Sclerosing Cholangitis [Internet]. In: Bhat PP, Dretler AA, Gdowski MM, Ramgopal RR, Williams DD, editors. Washington Manual of Medical Therapeutics. Wolters Kluwer Health; 2016. [cited 2023 June 04]. Available from: https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602011/all/Primary_Sclerosing_Cholangitis.
* Article titles in AMA citation format should be in sentence-case
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T1 - Primary Sclerosing Cholangitis
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ED - Dretler,Alexandra,
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ED - Ramgopal,Rajeev,
BT - Washington Manual of Medical Therapeutics
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PB - Wolters Kluwer Health
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