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Differential Diagnosis from Primary Sclerosing Cholangitis

  • Sung-Hoon Moon
  • Myung-Hwan Kim
Chapter

Abstract

Primary sclerosing cholangitis (PSC), a chronic progressive disease of unknown etiology, is characterized by fibrosis and strictures involving the intra- and extrahepatic bile ducts [1, 2]. PSC is a distinct entity from IgG4-related sclerosing cholangitis (IgG4-SC), but some IgG4-SC masquerades as PSC by way of similar manifestations, such as frequent stenosis of both intra- and extrahepatic bile ducts, bile ductal wall thickening, male predominance, cholestatic liver dysfunction, and initial mild symptoms [3–7]. The differential diagnosis between PSC and IgG4-SC is clinically important because their treatment modalities and prognosis are very different [2, 7, 8]. Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate corticosteroid therapy that can reverse bile duct strictures/wall thickening and cholestatic liver dysfunction and could potentially prevent future advanced liver disease [2]. A proper diagnosis of PSC, in turn, is crucial for optimizing the surveillance of the disease progression to hepatic decompensation and the need for liver transplantation.

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Copyright information

© Springer Science+Business Media Singapore 2019

Authors and Affiliations

  1. 1.Department of Internal MedicineHallym University College of Medicine, Hallym University Sacred Heart HospitalAnyangSouth Korea
  2. 2.Department of Internal MedicineUniversity of Ulsan College of Medicine, Asan Medical CenterSeoulSouth Korea

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