A condition closely mimicking IgG4-related disease despite the absence of serum IgG4 elevation and IgG4-positive plasma cell infiltration

Mod Rheumatol. 2016 Sep;26(5):784-9. doi: 10.3109/14397595.2014.916836.

Abstract

We describe a 74-year-old Japanese man with systemic fibroinflammatory conditions closely resembling those of immunoglobulin G4-related disease (IgG4-RD). Radiology and histology showed characteristics of IgG4-related tubulointerstitial nephritis, despite normal serum IgG4 value and scanty IgG4-positive plasma cell infiltration in each organ. This case suggests that a condition closely mimicking IgG4-RD may develop without IgG4-positive plasma cells and those exceptional cases should also be taken into account in the differential diagnosis of IgG4-RD.

Keywords: IgG4; IgG4-related disease; Tubulointerstitial nephritis.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / diagnosis*
  • Diagnosis, Differential
  • Humans
  • Immunoglobulin G / blood*
  • Male
  • Nephritis, Interstitial / blood
  • Nephritis, Interstitial / diagnosis*
  • Nephritis, Interstitial / pathology
  • Plasma Cells / immunology
  • Plasma Cells / pathology*

Substances

  • Immunoglobulin G