Clinical and Experimental Nephrology

, Volume 12, Issue 6, pp 421–431 | Cite as

Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan

  • Kensuke Joh
  • Takashi Taguchi
  • Hidekazu Shigematsu
  • Yutaka Kobayashi
  • Hiroshi Sato
  • Shinichi Nishi
  • Ritsuko Katafuchi
  • Shinsuke Nomura
  • Yoshihide Fujigaki
  • Yasunori Utsunomiya
  • Hitoshi Sugiyama
  • Takao Saito
  • Hirofumi Makino
Original Article Special issue Podocytic infolding glomerulopathy: a proposed new disease entity

Abstract

Background

A rare and peculiar glomerulopathy has begun to be recognized in Japan. The Japanese Society of Nephrology has established a research working group and has collected cases from all over Japan in an attempt to understand the complete spectrum of this glomerulopathy.

Method

The diagnostic criterion, which was needed to collect the cases, was proposed as a glomerulopathy showing microspheres or microtubular structures or both associated with podocytic infolding into the glomerular basement membrane (GBM) on electron microscopy. The lesion shows a non-argentaffin hole in the GBM with periodic acid methenamine silver staining and is similar to membranous glomerulonephritis.

Results

Twenty-five cases were collected from 17 institutions. Patients were 20–69 years old (19 women, 6 men). Seventeen patients also had collagen diseases such as lupus nephritis and Sjögren’s syndrome. All patients had proteinuria. Proteinuria showed a remission in 15 of 23 patients within 12 months, but proteinuria remained higher than 1.0 g/day in five patients despite different types of therapy. Podocytic infolding including microspheres showed either positive or negative staining for immunoglobulins. Cluster formation of microspheres was found in 4 of 17 patients with collagen disease, and in five out eight patients without collagen disease. Electron-dense deposits in the GBM were also found in 6 of 17 patients with collagen disease but were not found in eight patients without collagen disease.

Conclusion

Some patients might have a subtype of lupus nephritis, class V, or membranous glomerulonephritis. However, we propose a new disease entity, podocytic infolding glomerulopathy, as a common basis of all 25 patients, because we suspect that microspheres or microtubular structures or both can be derived from podocytic infolding.

Keywords

Renal biopsy Glomerulonephritis Podocytic infolding Electron microscopy Microspheres 

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Copyright information

© Japanese Society of Nephrology 2008

Authors and Affiliations

  • Kensuke Joh
    • 1
  • Takashi Taguchi
    • 2
  • Hidekazu Shigematsu
    • 3
  • Yutaka Kobayashi
    • 4
  • Hiroshi Sato
    • 5
  • Shinichi Nishi
    • 6
  • Ritsuko Katafuchi
    • 7
  • Shinsuke Nomura
    • 8
  • Yoshihide Fujigaki
    • 9
  • Yasunori Utsunomiya
    • 10
  • Hitoshi Sugiyama
    • 11
  • Takao Saito
    • 12
  • Hirofumi Makino
    • 11
  1. 1.Division of Renal Pathology, Clinical Research CenterChiba-East National HospitalChibaJapan
  2. 2.Department of PathologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
  3. 3.Department of HistopathologyShinshu University School of MedicineMatsumotoJapan
  4. 4.Division of NephrologyAkebono HospitalTokyoJapan
  5. 5.Department of Nephrology, Hypertension, EndocrinologyTohoku University Graduate School of MedicineSendaiJapan
  6. 6.Division of Clinical Nephrology and RheumatologyGraduate School of Medical and Dental Sciences, Niigata UniversityNiigataJapan
  7. 7.Division of Internal MedicineNational Fukuoka-Higashi Medical CenterFukuokaJapan
  8. 8.Department of Cardiology and NephrologyMie University Graduate School of MedicineMieJapan
  9. 9.The First Department of MedicineHamamatsu University School of MedicineHamamatsuJapan
  10. 10.Division of Kidney and HypertensionThe Jikei University School of MedicineTokyoJapan
  11. 11.Department of Medicine and Clinical ScienceOkayama University Graduate School of Medicine, Dentistry and Pharmaceutical SciencesOkayamaJapan
  12. 12.Division of Nephrology and Rheumatology, Department of Internal MedicineFukuoka University School of MedicineFukuokaJapan

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