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IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study
BACKGROUND: IgM nephropathy is a rare disease with variable clinical presentations and is an unusual cause of nephrotic syndrome. Histopathological findings typically include mesangial hypercellularity with IgM and complement deposition, though the spectrum may range from normal glomeruli through to...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7487712/ https://www.ncbi.nlm.nih.gov/pubmed/32894094 http://dx.doi.org/10.1186/s12882-020-02048-5 |
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author | Downie, Elizabeth Diep, Jason Sungala, Nagendraprasad Wong, Jeffrey |
author_facet | Downie, Elizabeth Diep, Jason Sungala, Nagendraprasad Wong, Jeffrey |
author_sort | Downie, Elizabeth |
collection | PubMed |
description | BACKGROUND: IgM nephropathy is a rare disease with variable clinical presentations and is an unusual cause of nephrotic syndrome. Histopathological findings typically include mesangial hypercellularity with IgM and complement deposition, though the spectrum may range from normal glomeruli through to focal and segmental glomerulosclerosis. Thromboembolism is a well recognised complication of nephrotic syndrome, but cerebral venous sinus thrombosis is rarely described. CASE PRESENTATION: This is the case of a 23-year-old male presenting with the nephrotic syndrome, whose initial renal biopsy was consistent with minimal change disease. Complete remission was achieved with prednisone, however multiple relapses and steroid dependence prompted re-biopsy, the results of which were more consistent with IgM nephropathy. His last relapse was complicated by cerebral venous sinus thrombosis. He then received rituximab and a weaning course of prednisone to again enter remission. CONCLUSIONS: This case highlights the need to consider IgM nephropathy in the differential diagnosis of nephrotic syndrome. Additionally, it emphasises the risk of thrombosis in patients with severe nephrosis. |
format | Online Article Text |
id | pubmed-7487712 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-74877122020-09-16 IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study Downie, Elizabeth Diep, Jason Sungala, Nagendraprasad Wong, Jeffrey BMC Nephrol Case Report BACKGROUND: IgM nephropathy is a rare disease with variable clinical presentations and is an unusual cause of nephrotic syndrome. Histopathological findings typically include mesangial hypercellularity with IgM and complement deposition, though the spectrum may range from normal glomeruli through to focal and segmental glomerulosclerosis. Thromboembolism is a well recognised complication of nephrotic syndrome, but cerebral venous sinus thrombosis is rarely described. CASE PRESENTATION: This is the case of a 23-year-old male presenting with the nephrotic syndrome, whose initial renal biopsy was consistent with minimal change disease. Complete remission was achieved with prednisone, however multiple relapses and steroid dependence prompted re-biopsy, the results of which were more consistent with IgM nephropathy. His last relapse was complicated by cerebral venous sinus thrombosis. He then received rituximab and a weaning course of prednisone to again enter remission. CONCLUSIONS: This case highlights the need to consider IgM nephropathy in the differential diagnosis of nephrotic syndrome. Additionally, it emphasises the risk of thrombosis in patients with severe nephrosis. BioMed Central 2020-09-07 /pmc/articles/PMC7487712/ /pubmed/32894094 http://dx.doi.org/10.1186/s12882-020-02048-5 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Downie, Elizabeth Diep, Jason Sungala, Nagendraprasad Wong, Jeffrey IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title | IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_full | IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_fullStr | IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_full_unstemmed | IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_short | IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_sort | igm nephropathy complicated by cerebral venous sinus thrombosis: a case study |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7487712/ https://www.ncbi.nlm.nih.gov/pubmed/32894094 http://dx.doi.org/10.1186/s12882-020-02048-5 |
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