TY - JOUR
T1 - Proposal of podocytic infolding glomerulopathy as a new disease entity
T2 - A review of 25 cases from nationwide research in Japan
AU - Joh, Kensuke
AU - Taguchi, Takashi
AU - Shigematsu, Hidekazu
AU - Kobayashi, Yutaka
AU - Sato, Hiroshi
AU - Nishi, Shinichi
AU - Katafuchi, Ritsuko
AU - Nomura, Shinsuke
AU - Fujigaki, Yoshihide
AU - Utsunomiya, Yasunori
AU - Sugiyama, Hitoshi
AU - Saito, Takao
AU - Makino, Hirofumi
PY - 2008/12
Y1 - 2008/12
N2 - 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.
AB - 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.
KW - Electron microscopy
KW - Glomerulonephritis
KW - Microspheres
KW - Podocytic infolding
KW - Renal biopsy
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U2 - 10.1007/s10157-008-0104-z
DO - 10.1007/s10157-008-0104-z
M3 - Article
C2 - 19012046
AN - SCOPUS:56649092727
VL - 12
SP - 421
EP - 431
JO - Clinical and Experimental Nephrology
JF - Clinical and Experimental Nephrology
SN - 1342-1751
IS - 6
ER -