Differential Diagnosis Between Proximal-Type IgG4-Related Sclerosing Cholangitis and Hilar Cholangiocarcinoma

  • Kensuke KubotaEmail author
  • Akito Iwasaki
  • Takamitsu Sato
  • Kunihiro Hosono


Indeterminant hilar biliary strictures (IHB) such as IgG4-related sclerosing cholangitis (IgG4-SC) and Klatskin tumor are leading causes and needed to differentiate from each other. Majority of IgG4-SC can be diagnosed without difficulty according to the JHBPS criterion 2012 [1] based on Nakazawa classification [2]. IgG4-SC patients are often associated with other organ involvements such as the pancreas (AIP), major salivary grounds, and/or retroperitoneal organs [1]. IgG4-SC can be divided into two types: the major is IgG4-SC associated with AIP (AIP-SC), and the minor is isolated IgG4-SC (i-SC). Moreover, i-SC consists of intrahepatic/hilar type (proximal, i-SC) and intrapancreatic type, and the characteristic features were still unknown. Proximal-type IgG4-SC, especially Klatskin tumor mimicker, should be excluded because it could be controlled by steroid treatment; besides, Klatskin tumor would be treated by radical surgery or poor prognosis after conservative treatment [3]. Most proximal-type IgG4-SC are associated with AIP; on the other hand, i-SC is cumbersome to rule out cancer. Some proximal IgG4-SC developed as recurrence after remission of AIP. IgG4-SC affects deep lesion of mucosa beyond the epithelium with abundant IgG4-positive lymphoplasma cell infiltration with lateral lesion; besides, Klatskin tumor invades through the mucosa sometimes associated with skip lesion (Fig. 13.1). As endoscopic biopsy tried to detect cancer in the diagnosis of IgG4-SC, it could not evaluate and diagnose IgG4-SC because true lesion is located further in the deep lesion, in which biopsy could not reach the lesion under the mucosa [4]. Steroid trial would be a better option under the situation [1]. This article showed the tips on how to differentiate indeterminant strictures; IgG4-SC and Klastkin tumor in especially on the view point of imaging diagnosis and supplement role of steroid trial.


IgG4-SC AIP Isolated IgG4-SC Klatskin tumor Steroid trial 


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Copyright information

© Springer Science+Business Media Singapore 2019

Authors and Affiliations

  • Kensuke Kubota
    • 1
    Email author
  • Akito Iwasaki
    • 1
  • Takamitsu Sato
    • 1
  • Kunihiro Hosono
    • 1
  1. 1.Endoscopic UnityYokohama City University HospitalYokohamaJapan

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