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The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy

The first case of eosinophilic granulomatosis with polyangiitis (EGPA) simultaneously demonstrating various clinical manifestations, including retroperitoneal fibrosis (RPF) causing hydronephrosis and membranous nephropathy (MN) leading to nephrotic syndrome, is presented. There have been no previou...

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Autores principales: Kondo, Isao, Arai, Yohei, Sakamoto, Emi, Katagiri, Daisuke, Hinoshita, Fumihiko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8263185/
https://www.ncbi.nlm.nih.gov/pubmed/33518573
http://dx.doi.org/10.2169/internalmedicine.6399-20
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author Kondo, Isao
Arai, Yohei
Sakamoto, Emi
Katagiri, Daisuke
Hinoshita, Fumihiko
author_facet Kondo, Isao
Arai, Yohei
Sakamoto, Emi
Katagiri, Daisuke
Hinoshita, Fumihiko
author_sort Kondo, Isao
collection PubMed
description The first case of eosinophilic granulomatosis with polyangiitis (EGPA) simultaneously demonstrating various clinical manifestations, including retroperitoneal fibrosis (RPF) causing hydronephrosis and membranous nephropathy (MN) leading to nephrotic syndrome, is presented. There have been no previous case reports demonstrating the simultaneous onset of these three disease categories with significant complex pathologies. This case was successfully managed by providing adequate combination therapies according to each disease category, leading to complete remission (CR) of all three diseases. In conclusion, we believe this case is extremely rare and clinically suggestive, and that these findings can be applied to a future phenotype-tailored treatment strategy for EGPA.
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spelling pubmed-82631852021-07-12 The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy Kondo, Isao Arai, Yohei Sakamoto, Emi Katagiri, Daisuke Hinoshita, Fumihiko Intern Med Case Report The first case of eosinophilic granulomatosis with polyangiitis (EGPA) simultaneously demonstrating various clinical manifestations, including retroperitoneal fibrosis (RPF) causing hydronephrosis and membranous nephropathy (MN) leading to nephrotic syndrome, is presented. There have been no previous case reports demonstrating the simultaneous onset of these three disease categories with significant complex pathologies. This case was successfully managed by providing adequate combination therapies according to each disease category, leading to complete remission (CR) of all three diseases. In conclusion, we believe this case is extremely rare and clinically suggestive, and that these findings can be applied to a future phenotype-tailored treatment strategy for EGPA. The Japanese Society of Internal Medicine 2021-02-01 2021-06-15 /pmc/articles/PMC8263185/ /pubmed/33518573 http://dx.doi.org/10.2169/internalmedicine.6399-20 Text en Copyright © 2021 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Kondo, Isao
Arai, Yohei
Sakamoto, Emi
Katagiri, Daisuke
Hinoshita, Fumihiko
The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy
title The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy
title_full The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy
title_fullStr The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy
title_full_unstemmed The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy
title_short The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy
title_sort first case of eosinophilic granulomatosis with polyangiitis simultaneously demonstrating various clinical manifestations with retroperitoneal fibrosis and membranous nephropathy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8263185/
https://www.ncbi.nlm.nih.gov/pubmed/33518573
http://dx.doi.org/10.2169/internalmedicine.6399-20
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