IgG4-Related Kidney Disease

  • Takako Saeki
  • Mitsuhiro Kawano
  • Kazuhiro Yoshita
  • Mitsuhiro Ueno
  • Michio Nagata
  • Yutaka Yamaguchi


The main pathological finding in IgG4-related kidney disease (IgG4-RKD) is a form of tubulointerstitial nephritis (TIN), characterized by a dense lymphoplasmacytic infiltrate associated with abundant IgG4-positive plasma cells and fibrosis. Storiform fibrosis, the pattern of fibrosis often observed in IgG4-related TIN, is rarely associated with TIN of other causes, making it a useful differentiating feature. Other findings suggestive of IgG4-related TIN are as follows: clear demarcation between the affected and unaffected portions; extension of inflammatory cell infiltrates beyond the renal capsule; and eosinophil infiltration. By immunofluorescence and electron microscopy, immune complex deposits (or electron-dense deposits) are shown in tubular basement membrane in many cases. Although glomeruli generally show minor abnormalities, some glomerular lesions suggestive of immune complex involvement are also occasionally present. Among these glomerular lesions, membranous nephropathy and Henoch–Schönlein purpura nephritis have attracted the most attention. Further consideration must be given to these lesions as they may yield insights about the pathogenesis of IgG4-RKD. A goal of future work in IgG4-RKD is more extensive elucidation of the relationship between TIN and the glomerular lesions of IgG4-RKD.


Membranous Nephropathy Glomerular Lesion Immune Complex Deposit Idiopathic Membranous Nephropathy Tubular Basement Membrane 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Japan 2014

Authors and Affiliations

  • Takako Saeki
    • 1
  • Mitsuhiro Kawano
    • 2
  • Kazuhiro Yoshita
    • 3
  • Mitsuhiro Ueno
    • 4
  • Michio Nagata
    • 5
  • Yutaka Yamaguchi
    • 6
  1. 1.Department of Internal MedicineNagaoka Red Cross HospitalNagaoka cityJapan
  2. 2.Division of RheumatologyKanazawa University HospitalKanazawaJapan
  3. 3.Division of Clinical Nephrology and RheumatologyNiigata University Graduate School of Medical and Dental SciencesNiigataJapan
  4. 4.University Health Center, Joetsu University of EducationJoetsuJapan
  5. 5.Department of Kidney and Vascular Pathology, Graduate School of Comprehensive Human SciencesUniversity of TsukubaTsukubaJapan
  6. 6.Yamaguchi’s Pathology LaboratoryChibaJapan

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