A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis

A 58-year-old man with type 1 autoimmune pancreatitis was referred to nephrologists for severe proteinuria. Laboratory data revealed a high serum IgG4 level, hypoalbuminemia, and massive proteinuria, which were compatible with nephrotic syndrome. The renal pathological findings confirmed the diagnos...

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Autores principales: Arai, Hiroyuki, Toda, Naohiro, Kamimatsuse, Ryo, Nishioka, Keisuke, Endo, Shuichiro, Akiyama, Shinichi, Maruyama, Shoichi, Matsubara, Takeshi, Yokoi, Hideki, Yanagita, Motoko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6207830/
https://www.ncbi.nlm.nih.gov/pubmed/29709946
http://dx.doi.org/10.2169/internalmedicine.0836-18
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author Arai, Hiroyuki
Toda, Naohiro
Kamimatsuse, Ryo
Nishioka, Keisuke
Endo, Shuichiro
Akiyama, Shinichi
Maruyama, Shoichi
Matsubara, Takeshi
Yokoi, Hideki
Yanagita, Motoko
author_facet Arai, Hiroyuki
Toda, Naohiro
Kamimatsuse, Ryo
Nishioka, Keisuke
Endo, Shuichiro
Akiyama, Shinichi
Maruyama, Shoichi
Matsubara, Takeshi
Yokoi, Hideki
Yanagita, Motoko
author_sort Arai, Hiroyuki
collection PubMed
description A 58-year-old man with type 1 autoimmune pancreatitis was referred to nephrologists for severe proteinuria. Laboratory data revealed a high serum IgG4 level, hypoalbuminemia, and massive proteinuria, which were compatible with nephrotic syndrome. The renal pathological findings confirmed the diagnosis of secondary membranous nephropathy concurrent with IgG4-related tubulointerstitial nephritis. Despite the improvement of interstitial markers, the proteinuria was refractory to prednisolone, requiring cyclosporine to achieve complete remission. Membranous nephropathy is a rare manifestation of IgG4-related kidney disease. This case shows that the therapeutic response to prednisolone significantly differs between glomerular lesions and interstitial lesions of IgG4-related kidney disease.
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spelling pubmed-62078302018-10-31 A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis Arai, Hiroyuki Toda, Naohiro Kamimatsuse, Ryo Nishioka, Keisuke Endo, Shuichiro Akiyama, Shinichi Maruyama, Shoichi Matsubara, Takeshi Yokoi, Hideki Yanagita, Motoko Intern Med Case Report A 58-year-old man with type 1 autoimmune pancreatitis was referred to nephrologists for severe proteinuria. Laboratory data revealed a high serum IgG4 level, hypoalbuminemia, and massive proteinuria, which were compatible with nephrotic syndrome. The renal pathological findings confirmed the diagnosis of secondary membranous nephropathy concurrent with IgG4-related tubulointerstitial nephritis. Despite the improvement of interstitial markers, the proteinuria was refractory to prednisolone, requiring cyclosporine to achieve complete remission. Membranous nephropathy is a rare manifestation of IgG4-related kidney disease. This case shows that the therapeutic response to prednisolone significantly differs between glomerular lesions and interstitial lesions of IgG4-related kidney disease. The Japanese Society of Internal Medicine 2018-04-27 2018-10-01 /pmc/articles/PMC6207830/ /pubmed/29709946 http://dx.doi.org/10.2169/internalmedicine.0836-18 Text en Copyright © 2018 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access article 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
Arai, Hiroyuki
Toda, Naohiro
Kamimatsuse, Ryo
Nishioka, Keisuke
Endo, Shuichiro
Akiyama, Shinichi
Maruyama, Shoichi
Matsubara, Takeshi
Yokoi, Hideki
Yanagita, Motoko
A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis
title A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis
title_full A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis
title_fullStr A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis
title_full_unstemmed A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis
title_short A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis
title_sort refractory case of secondary membranous nephropathy concurrent with igg4-related tubulointerstitial nephritis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6207830/
https://www.ncbi.nlm.nih.gov/pubmed/29709946
http://dx.doi.org/10.2169/internalmedicine.0836-18
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