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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Japan
2014
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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 |
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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 |
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