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Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy

We present a 77-year-old Caucasian woman who presented with nephrotic-range proteinuria, microhematuria, renal impairment, and extremely elevated blood pressure. She had a long history of well-controlled type 2 diabetes. Renal biopsy revealed fibrillary deposits in the mesangium and glomerular basem...

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Autores principales: Tsui, Calvin, Dokouhaki, Pouneh, Prasad, Bhanu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6167697/
https://www.ncbi.nlm.nih.gov/pubmed/30320122
http://dx.doi.org/10.1159/000492529
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author Tsui, Calvin
Dokouhaki, Pouneh
Prasad, Bhanu
author_facet Tsui, Calvin
Dokouhaki, Pouneh
Prasad, Bhanu
author_sort Tsui, Calvin
collection PubMed
description We present a 77-year-old Caucasian woman who presented with nephrotic-range proteinuria, microhematuria, renal impairment, and extremely elevated blood pressure. She had a long history of well-controlled type 2 diabetes. Renal biopsy revealed fibrillary deposits in the mesangium and glomerular basement membrane consistent with fibrillary glomerulopathy (FGN), with crescentic changes and thrombotic microangiopathy (TMA). We could not identify any radiological, clinical, or laboratory evidence of autoimmune disorders, lymphoproliferative disorders, and malignancy. It was decided not to offer her any immunosuppressive therapy, as she was frail with substantial renal damage on the biopsy. Five months after presentation, she gradually progressed to requiring renal replacement therapy and is currently on maintenance hemodialysis. Crescentic changes in FGN, though rare, have been previously described, but the concurrent presence of TMA has never been previously reported.
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spelling pubmed-61676972018-10-12 Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy Tsui, Calvin Dokouhaki, Pouneh Prasad, Bhanu Case Rep Nephrol Dial Case Report We present a 77-year-old Caucasian woman who presented with nephrotic-range proteinuria, microhematuria, renal impairment, and extremely elevated blood pressure. She had a long history of well-controlled type 2 diabetes. Renal biopsy revealed fibrillary deposits in the mesangium and glomerular basement membrane consistent with fibrillary glomerulopathy (FGN), with crescentic changes and thrombotic microangiopathy (TMA). We could not identify any radiological, clinical, or laboratory evidence of autoimmune disorders, lymphoproliferative disorders, and malignancy. It was decided not to offer her any immunosuppressive therapy, as she was frail with substantial renal damage on the biopsy. Five months after presentation, she gradually progressed to requiring renal replacement therapy and is currently on maintenance hemodialysis. Crescentic changes in FGN, though rare, have been previously described, but the concurrent presence of TMA has never been previously reported. S. Karger AG 2018-08-31 /pmc/articles/PMC6167697/ /pubmed/30320122 http://dx.doi.org/10.1159/000492529 Text en Copyright © 2018 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Case Report
Tsui, Calvin
Dokouhaki, Pouneh
Prasad, Bhanu
Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy
title Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy
title_full Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy
title_fullStr Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy
title_full_unstemmed Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy
title_short Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy
title_sort fibrillary glomerulonephritis with crescentic and necrotizing glomerulonephritis and concurrent thrombotic microangiopathy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6167697/
https://www.ncbi.nlm.nih.gov/pubmed/30320122
http://dx.doi.org/10.1159/000492529
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