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IgG4-related tubulointerstitial nephritis accompanied with cystic formation

BACKGROUND: An immunoglobulin G4 (IgG4)-related disease is important disease in differential diagnosis of tumors in kidney, pancreas, lung and other organs. The imaging findings of IgG4-related kidney diseases are usually expressed as defect contrast region, while cystic formation in kidney is extre...

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Autores principales: Fukuhara, Hideo, Taniguchi, Yoshinori, Matsumoto, Manabu, Kuroda, Naoto, Fukata, Satoshi, Inoue, Keiji, Fujimoto, Shimpei, Terada, Yoshio, Shuin, Taro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4107474/
https://www.ncbi.nlm.nih.gov/pubmed/25038818
http://dx.doi.org/10.1186/1471-2490-14-54
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author Fukuhara, Hideo
Taniguchi, Yoshinori
Matsumoto, Manabu
Kuroda, Naoto
Fukata, Satoshi
Inoue, Keiji
Fujimoto, Shimpei
Terada, Yoshio
Shuin, Taro
author_facet Fukuhara, Hideo
Taniguchi, Yoshinori
Matsumoto, Manabu
Kuroda, Naoto
Fukata, Satoshi
Inoue, Keiji
Fujimoto, Shimpei
Terada, Yoshio
Shuin, Taro
author_sort Fukuhara, Hideo
collection PubMed
description BACKGROUND: An immunoglobulin G4 (IgG4)-related disease is important disease in differential diagnosis of tumors in kidney, pancreas, lung and other organs. The imaging findings of IgG4-related kidney diseases are usually expressed as defect contrast region, while cystic formation in kidney is extremely rare. Here, we report a case of IgG4-related tubulointerstitial nephritis with renal cystic change caused by the narrowing or obstruction of collecting duct in renal medulla. CASE PRESENTATION: Abdominal contrasted CT scan showed a 31 × 24 mm cystic tumor at the upper pole of the right kidney and multiple low-attenuation areas in the left kidney. (18) F-fluorodeoxyglucose (FDG)-PET/CT scan showed moderate FDG accumulation of cystic tumor in marginal lesion. In addition, FDG-PET/CT scan also showed moderate FDG accumulation in the pancreatic body. Laparoscopic right nephrectomy was performed. Histological examination was revealed lymphoplasmacytic infiltrate with focal fibrosis and severe narrowing or obstruction of lumen of collecting duct in renal medulla. Furthermore, the IgG4 positive plasma cells infiltrated exceeding 10 cells per one high-power field in renal medulla. The ratio of IgG4-plasma cells to IgG-positive plasma cells was about 50%. The serum level of IgG4 was also elevated (218 mg/dl). Based on these findings, we finally diagnosed IgG4-related tubulointerstitial nephritis with renal cystic change. CONCLUSION: IgG4-related kidney disease might cause cystic formation by severe narrowing and obstruction of collecting duct.
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spelling pubmed-41074742014-07-24 IgG4-related tubulointerstitial nephritis accompanied with cystic formation Fukuhara, Hideo Taniguchi, Yoshinori Matsumoto, Manabu Kuroda, Naoto Fukata, Satoshi Inoue, Keiji Fujimoto, Shimpei Terada, Yoshio Shuin, Taro BMC Urol Case Report BACKGROUND: An immunoglobulin G4 (IgG4)-related disease is important disease in differential diagnosis of tumors in kidney, pancreas, lung and other organs. The imaging findings of IgG4-related kidney diseases are usually expressed as defect contrast region, while cystic formation in kidney is extremely rare. Here, we report a case of IgG4-related tubulointerstitial nephritis with renal cystic change caused by the narrowing or obstruction of collecting duct in renal medulla. CASE PRESENTATION: Abdominal contrasted CT scan showed a 31 × 24 mm cystic tumor at the upper pole of the right kidney and multiple low-attenuation areas in the left kidney. (18) F-fluorodeoxyglucose (FDG)-PET/CT scan showed moderate FDG accumulation of cystic tumor in marginal lesion. In addition, FDG-PET/CT scan also showed moderate FDG accumulation in the pancreatic body. Laparoscopic right nephrectomy was performed. Histological examination was revealed lymphoplasmacytic infiltrate with focal fibrosis and severe narrowing or obstruction of lumen of collecting duct in renal medulla. Furthermore, the IgG4 positive plasma cells infiltrated exceeding 10 cells per one high-power field in renal medulla. The ratio of IgG4-plasma cells to IgG-positive plasma cells was about 50%. The serum level of IgG4 was also elevated (218 mg/dl). Based on these findings, we finally diagnosed IgG4-related tubulointerstitial nephritis with renal cystic change. CONCLUSION: IgG4-related kidney disease might cause cystic formation by severe narrowing and obstruction of collecting duct. BioMed Central 2014-07-20 /pmc/articles/PMC4107474/ /pubmed/25038818 http://dx.doi.org/10.1186/1471-2490-14-54 Text en Copyright © 2014 Fukuhara et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. 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.
spellingShingle Case Report
Fukuhara, Hideo
Taniguchi, Yoshinori
Matsumoto, Manabu
Kuroda, Naoto
Fukata, Satoshi
Inoue, Keiji
Fujimoto, Shimpei
Terada, Yoshio
Shuin, Taro
IgG4-related tubulointerstitial nephritis accompanied with cystic formation
title IgG4-related tubulointerstitial nephritis accompanied with cystic formation
title_full IgG4-related tubulointerstitial nephritis accompanied with cystic formation
title_fullStr IgG4-related tubulointerstitial nephritis accompanied with cystic formation
title_full_unstemmed IgG4-related tubulointerstitial nephritis accompanied with cystic formation
title_short IgG4-related tubulointerstitial nephritis accompanied with cystic formation
title_sort igg4-related tubulointerstitial nephritis accompanied with cystic formation
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4107474/
https://www.ncbi.nlm.nih.gov/pubmed/25038818
http://dx.doi.org/10.1186/1471-2490-14-54
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