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Primary Sclerosing Cholangitis: A Concise Review of Diagnosis and Management

  • Zachary P. FrickerEmail author
  • David R. Lichtenstein
Mentored Reviews

Abstract

Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterized by progressive idiopathic stricturing of the biliary system, typically leading to cirrhosis, end-stage liver disease, and colonic or hepatobiliary malignancy. Its presentation is often that of asymptomatic alkaline phosphatase elevation. When symptoms are present, they typically include fatigue, pruritus, or jaundice. The diagnosis can be confirmed via cholangiography, either magnetic resonance cholangiography (MRCP) or endoscopic retrograde cholangiography if the former is inconclusive. The clinical course is marked by progressive liver disease leading to cirrhosis with its attendant complications of portal hypertension, often including recurrent episodes of cholangitis. Greater elevation in alkaline phosphatase or liver stiffness is associated with worse clinical outcomes. Management includes endoscopic treatment of symptomatic biliary strictures and evaluation of dominant strictures as no adequate medical treatment is available. Multiple medical therapies are under evaluation. Ultimately, liver transplantation may be necessary for management of decompensated cirrhosis or disabling symptoms. There is also a markedly increased risk of cancer, notably including cholangiocarcinoma and gallbladder and colorectal cancers (particularly in patients with colitis). Cancer screening can be done with semi-annual liver imaging (MRCP or ultrasound) and colonoscopy every 1–2 years in those with colitis.

Keywords

Primary sclerosing cholangitis (PSC) Cholangiocarcinoma Cholangiopathy Cholestasis 

Notes

Funding

Dr. Fricker was supported in part by the Boston University Clinical and Translational Science Institute (Grant UL1-TR000157).

Compliance with ethical standards

Conflict of interest

Dr. Lichtenstein has received a speaker’s honorarium from Aries Pharmaceutical, GI Supply, and Olympus (for whom he also serves as a consultant) and research grants from Iterative Scopes (for whom he also serves as a consultant) and Motus. He is a member of the ABIM Exam Committee for Gastroenterology, the ASGE Technology committee, and a Clinical Events Committee for Augmenix.

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of GastroenterologyUniversity of Pennsylvania Perelman School of MedicinePhiladelphiaUSA
  2. 2.Section of Gastroenterology, Evans Department of MedicineBoston University School of MedicineBostonUSA

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