Cargando…

An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?

Lupus nephritis (LN) is usually associated with immune deposition in the glomerular capillary wall. On the other hand, focal segmental glomerulosclerosis (FSGS) is not typically associated with immune deposition, and its pathogenesis includes podocyte damage and loss. The definition of lupus podocyt...

Descripción completa

Detalles Bibliográficos
Autores principales: Hanaoka, Hironari, Hashiguchi, Akinori, Konishi, Konosuke, Kuwana, Masataka, Takeuchi, Tsutomu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Japan 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5411626/
https://www.ncbi.nlm.nih.gov/pubmed/28509272
http://dx.doi.org/10.1007/s13730-014-0142-1
_version_ 1783232846113013760
author Hanaoka, Hironari
Hashiguchi, Akinori
Konishi, Konosuke
Kuwana, Masataka
Takeuchi, Tsutomu
author_facet Hanaoka, Hironari
Hashiguchi, Akinori
Konishi, Konosuke
Kuwana, Masataka
Takeuchi, Tsutomu
author_sort Hanaoka, Hironari
collection PubMed
description Lupus nephritis (LN) is usually associated with immune deposition in the glomerular capillary wall. On the other hand, focal segmental glomerulosclerosis (FSGS) is not typically associated with immune deposition, and its pathogenesis includes podocyte damage and loss. The definition of lupus podocytopathy (LP) excludes patients with electron-dense glomerular basement membrane deposits. Here, we report the case of an LN patient with nephrotic proteinuria. Renal pathology demonstrated focal endocapillary hypercellularity superimposed on foam cells. Immunofluorescence revealed diffuse global subepithelial immune deposits, and electron microscopy showed electron-dense glomerular basement membrane deposits and diffuse foot process effacement. Treatment with steroid and cyclosporine improved her proteinuria. Post-treatment renal re-biopsy revealed focal segmental sclerotic lesions closely resembling FSGS. These results indicate that the pathogenesis of this case may involve an FSGS-like condition or podocytopathic change. It is possible that careful examination would reveal podocytopathic changes other than LP in patients previously diagnosed as LN class III + V. Further investigations are needed to understand FSGS-like pathological changes accompanied with capillary immune deposits in LN.
format Online
Article
Text
id pubmed-5411626
institution National Center for Biotechnology Information
language English
publishDate 2014
publisher Springer Japan
record_format MEDLINE/PubMed
spelling pubmed-54116262017-05-03 An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy? Hanaoka, Hironari Hashiguchi, Akinori Konishi, Konosuke Kuwana, Masataka Takeuchi, Tsutomu CEN Case Rep Case Report Lupus nephritis (LN) is usually associated with immune deposition in the glomerular capillary wall. On the other hand, focal segmental glomerulosclerosis (FSGS) is not typically associated with immune deposition, and its pathogenesis includes podocyte damage and loss. The definition of lupus podocytopathy (LP) excludes patients with electron-dense glomerular basement membrane deposits. Here, we report the case of an LN patient with nephrotic proteinuria. Renal pathology demonstrated focal endocapillary hypercellularity superimposed on foam cells. Immunofluorescence revealed diffuse global subepithelial immune deposits, and electron microscopy showed electron-dense glomerular basement membrane deposits and diffuse foot process effacement. Treatment with steroid and cyclosporine improved her proteinuria. Post-treatment renal re-biopsy revealed focal segmental sclerotic lesions closely resembling FSGS. These results indicate that the pathogenesis of this case may involve an FSGS-like condition or podocytopathic change. It is possible that careful examination would reveal podocytopathic changes other than LP in patients previously diagnosed as LN class III + V. Further investigations are needed to understand FSGS-like pathological changes accompanied with capillary immune deposits in LN. Springer Japan 2014-08-17 /pmc/articles/PMC5411626/ /pubmed/28509272 http://dx.doi.org/10.1007/s13730-014-0142-1 Text en © The Author(s) 2014 https://creativecommons.org/licenses/by/4.0/ Open AccessThis article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.
spellingShingle Case Report
Hanaoka, Hironari
Hashiguchi, Akinori
Konishi, Konosuke
Kuwana, Masataka
Takeuchi, Tsutomu
An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?
title An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?
title_full An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?
title_fullStr An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?
title_full_unstemmed An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?
title_short An unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?
title_sort unusual association between focal segmental sclerosis and lupus nephritis: a distinct concept from lupus podocytopathy?
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5411626/
https://www.ncbi.nlm.nih.gov/pubmed/28509272
http://dx.doi.org/10.1007/s13730-014-0142-1
work_keys_str_mv AT hanaokahironari anunusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT hashiguchiakinori anunusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT konishikonosuke anunusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT kuwanamasataka anunusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT takeuchitsutomu anunusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT hanaokahironari unusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT hashiguchiakinori unusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT konishikonosuke unusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT kuwanamasataka unusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy
AT takeuchitsutomu unusualassociationbetweenfocalsegmentalsclerosisandlupusnephritisadistinctconceptfromlupuspodocytopathy