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

研究成果: Article査読

19 被引用数 (Scopus)

抄録

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.

本文言語English
ページ(範囲)421-431
ページ数11
ジャーナルClinical and experimental nephrology
12
6
DOI
出版ステータスPublished - 2008 12
外部発表はい

ASJC Scopus subject areas

  • 生理学
  • 腎臓病学
  • 生理学(医学)

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