A Case of Hepatic Immunoglobulin G4-Related Disease Presenting as an Inflammatory Pseudotumor and Sclerosing Cholangitis

Se Young Jang, Young Seok Han, Sang Yub Lee, Ja Ryung Han, Young Oh Kweon, Won Young Tak, Soo Young Park, Yu Rim Lee, Hun Kyu Ryeom, Jung Guen Cha, Jihoon Hong, Yoo Na Kang

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3 Scopus citations

Abstract

An inflammatory pseudotumor is a benign disease characterized by tumor-like lesions consisting of inflammatory cells including plasma cells and fibrous tissue. Recently, some inflammatory pseudotumor cases proved to be a form of Immunoglobulin G4-related disease (IgG4-RD). This novel clinical entity, recognized as a fibroinflammatory condition, is characterized by lymphoplasmacytic infiltration with a predominance of IgG4-positive plasma cells, storiform fibrosis, and often elevated serum IgG4 concentrations. We report a case of IgG4-RD in the form of an inflammatory pseudotumor in the liver with combined sclerosing cholangitis. We recommend that for diagnosing IgG4-RD accurately, it is important to obtain adequate tissue samples and follow-up the lesion in clinical practice.

Original languageEnglish
Article number1497
JournalDiagnostics
Volume12
Issue number6
DOIs
StatePublished - Jun 2022

Keywords

  • biopsy
  • cholangitis
  • immunoglobulin G4-related disease
  • immunohistochemistry
  • liver
  • sclerosing
  • surgery

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