Clinical and Experimental Nephrology

, Volume 15, Issue 6, pp 810–819

Clinicopathological findings of immunoglobulin G4-related kidney disease

  • Shinichi Nishi
  • Naofumi Imai
  • Kazuhiro Yoshida
  • Yumi Ito
  • Takako Saeki
Review Article

DOI: 10.1007/s10157-011-0526-x

Cite this article as:
Nishi, S., Imai, N., Yoshida, K. et al. Clin Exp Nephrol (2011) 15: 810. doi:10.1007/s10157-011-0526-x

Abstract

Immunoglobulin (Ig) G4-related kidney disease characterizing tubulointerstitial nephritis (TIN) is an organ complication recognized in IgG4-related systemic diseases that has some unique aspects compared to other types of TIN. TIN lesions in the kidney can be tumor-like, focal or diffuse. Abnormal urinalysis is usually mild or absent even in the cases with deteriorated renal dysfunction. Some cases are accidentally diagnosed from radiological findings without renal dysfunction and/or abnormal urinalysis. The typical pathological findings of TIN are unique fibrosis and infiltration of massive lymphocytes and IgG4-positive plasma cells. Glomerular lesions are rare but the complication of mesangial proliferative glomerulonephritis and membranous nephropathy is occasionally reported. Pathogenic mechanisms are unclear until now; however, auto-immune and allergic mechanisms have been suspected from laboratory data. The initial response to steroid agents is generally favorable; however, recurrence is possible after the discontinuation of steroid treatment. Long-term follow-up is necessary with continuous systemic checks for organ disorders due to IgG4-related systemic diseases.

Keywords

Tubulointerstitial nephritisIgG4Plasma cellSteroid agent

Copyright information

© Japanese Society of Nephrology 2011

Authors and Affiliations

  • Shinichi Nishi
    • 1
  • Naofumi Imai
    • 2
  • Kazuhiro Yoshida
    • 2
  • Yumi Ito
    • 2
  • Takako Saeki
    • 3
  1. 1.Division of Nephrology and Kidney CenterKobe University Graduate School of MedicineKobeJapan
  2. 2.Division of Clinical Nephrology and RheumatologyNiigata University Graduate School of Medical and Dental SciencesNiigataJapan
  3. 3.Department of Internal MedicineNagaoka Red Cross HospitalNagaokaJapan