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

  • Original Article
  • Special issue Podocytic infolding glomerulopathy: a proposed new disease entity
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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.

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Authors and Affiliations

  1. Division of Renal Pathology, Clinical Research Center, Chiba-East National Hospital, 673 Nitona-cho, Chuo-ku, Chiba, 260-8712, Japan

    Kensuke Joh

  2. Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan

    Takashi Taguchi

  3. Department of Histopathology, Shinshu University School of Medicine, Matsumoto, Japan

    Hidekazu Shigematsu

  4. Division of Nephrology, Akebono Hospital, Tokyo, Japan

    Yutaka Kobayashi

  5. Department of Nephrology, Hypertension, Endocrinology, Tohoku University Graduate School of Medicine, Sendai, Japan

    Hiroshi Sato

  6. Division of Clinical Nephrology and Rheumatology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan

    Shinichi Nishi

  7. Division of Internal Medicine, National Fukuoka-Higashi Medical Center, Fukuoka, Japan

    Ritsuko Katafuchi

  8. Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Mie, Japan

    Shinsuke Nomura

  9. The First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan

    Yoshihide Fujigaki

  10. Division of Kidney and Hypertension, The Jikei University School of Medicine, Tokyo, Japan

    Yasunori Utsunomiya

  11. Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan

    Hitoshi Sugiyama & Hirofumi Makino

  12. Division of Nephrology and Rheumatology, Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka, Japan

    Takao Saito

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  1. Kensuke Joh
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  2. Takashi Taguchi
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Correspondence to Kensuke Joh.

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Joh, K., Taguchi, T., Shigematsu, H. et al. Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan. Clin Exp Nephrol 12, 421–431 (2008). https://doi.org/10.1007/s10157-008-0104-z

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  • DOI: https://doi.org/10.1007/s10157-008-0104-z

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