Diagnostics (Jun 2022)

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

DOI
https://doi.org/10.3390/diagnostics12061497
Journal volume & issue
Vol. 12, no. 6
p. 1497

Abstract

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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.

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