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Primary sclerosing cholangitis
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Primary sclerosing cholangitis

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Contributors: Michael W. Winter MD, Khaled Bittar MD, Nishant H. Patel MD, Desiree Rivera-Nieves MD
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Synopsis

Primary sclerosing cholangitis (PSC) is a chronic liver disease defined by inflammation and scarring of the bile ducts that may result in severe liver damage progressively over time. In addition to cirrhosis, patients with PSC are at high risk of developing recurrent biliary tract infections and at a higher risk of developing bile duct or liver tumors.

The etiology of PSC is unknown; it is associated with inflammatory bowel disease and is believed to be caused by a combination of genetic and environmental factors. PSC is most commonly diagnosed in patients around 40 years old, and 60% of patients are male. The highest incidences of PSC are in the United States and Northern Europe. Many patients are asymptomatic at time of diagnosis and early in the disease process, and PSC is detected by incidental liver test abnormalities. Common symptoms include pruritus, fatigue, and jaundice.

There is no specific approved therapy available for PSC and no cure besides liver transplantation. Goals of therapy focus on symptom management. There is mixed literature on the utility of ursodeoxycholic acid (ursodiol) and more recently obeticholic acid (US Food and Drug Administration [FDA] approved in 2016, unclear long-term benefit). However, the natural course of PSC is toward liver failure, with 40% of patients requiring a liver transplant. Prognosis for patients who have undergone liver transplantation is excellent, and a good quality of life should be expected after recovery, although disease recurrence is possible.

For more information, see OMIM.

Codes

ICD10CM:
K83.0 – Cholangitis

SNOMEDCT:
197441003 – Primary sclerosing cholangitis

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Last Reviewed: 11/09/2016
Last Updated: 03/29/2017
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Primary sclerosing cholangitis
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Primary sclerosing cholangitis : Fatigue, Hepatomegaly, Jaundice, Alkaline phosphatase elevated, Pruritus, RUQ pain
Copyright © 2019 VisualDx®. All rights reserved.