IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass

IgG4-related disease (IgG4-RD) is a nosological entity defined as a chronic immune-mediated fibro-inflammatory condition characterized by a tendency to form tumefactive, tissue-destructive lesions or by organ failure. Urologic involvement in IgG4-RD has been described in some short series of patient...

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Main Authors: Daniele Bianchi, Luca Topazio, Gabriele Gaziev, Valerio Iacovelli, Pierluigi Bove, Alessandro Mauriello, Enrico Finazzi Agrò
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Case Reports in Surgery
Online Access:http://dx.doi.org/10.1155/2017/9690218
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author Daniele Bianchi
Luca Topazio
Gabriele Gaziev
Valerio Iacovelli
Pierluigi Bove
Alessandro Mauriello
Enrico Finazzi Agrò
author_facet Daniele Bianchi
Luca Topazio
Gabriele Gaziev
Valerio Iacovelli
Pierluigi Bove
Alessandro Mauriello
Enrico Finazzi Agrò
author_sort Daniele Bianchi
collection DOAJ
description IgG4-related disease (IgG4-RD) is a nosological entity defined as a chronic immune-mediated fibro-inflammatory condition characterized by a tendency to form tumefactive, tissue-destructive lesions or by organ failure. Urologic involvement in IgG4-RD has been described in some short series of patients and in isolated case reports, most often involving the kidneys in so-called IgG4-related kidney disease (IgG4-RKD). The disease can occasionally mimic malignancies and is at risk of being misdiagnosed due to its rarity. We report the case of a 56-year-old man presenting with a right renal mass suspected of being malignant. Laboratory tests showed normal creatinine levels, a high erythrocyte sedimentation rate, and high levels of C-reactive protein and microalbuminuria. The patient underwent radical right nephroureterectomy and histopathologic examination revealed features proving IgG4-RKD. He was therefore referred to immunologists. Typical clinical presentation of IgG4-RKD includes altered renal function with inconstant or no radiologic findings. Conversely, in the case we presented, a single nodule was detected upon imaging evaluation, thus mimicking malignancy. This raises the issue of a proper differential diagnosis. A multidisciplinary approach can be useful, although in clinical practice the selection of patients suspected of having IgG4-RKD is critical in the cases presenting with a renal mass that mimics malignancy.
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spelling doaj-art-781fc0241e6a4f619c8c008ac250e76c2025-02-03T01:27:15ZengWileyCase Reports in Surgery2090-69002090-69192017-01-01201710.1155/2017/96902189690218IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal MassDaniele Bianchi0Luca Topazio1Gabriele Gaziev2Valerio Iacovelli3Pierluigi Bove4Alessandro Mauriello5Enrico Finazzi Agrò6Department of Urology, Policlinico Tor Vergata, Rome, ItalyDepartment of Urology, Policlinico Tor Vergata, Rome, ItalyDepartment of Urology, Policlinico Tor Vergata, Rome, ItalyDepartment of Urology, Policlinico Tor Vergata, Rome, ItalyDepartment of Urology, Policlinico Tor Vergata, Rome, ItalyAnatomic Pathology, Department of Experimental Medicine and Surgery, Tor Vergata University, Rome, ItalyDepartment of Urology, Policlinico Tor Vergata, Rome, ItalyIgG4-related disease (IgG4-RD) is a nosological entity defined as a chronic immune-mediated fibro-inflammatory condition characterized by a tendency to form tumefactive, tissue-destructive lesions or by organ failure. Urologic involvement in IgG4-RD has been described in some short series of patients and in isolated case reports, most often involving the kidneys in so-called IgG4-related kidney disease (IgG4-RKD). The disease can occasionally mimic malignancies and is at risk of being misdiagnosed due to its rarity. We report the case of a 56-year-old man presenting with a right renal mass suspected of being malignant. Laboratory tests showed normal creatinine levels, a high erythrocyte sedimentation rate, and high levels of C-reactive protein and microalbuminuria. The patient underwent radical right nephroureterectomy and histopathologic examination revealed features proving IgG4-RKD. He was therefore referred to immunologists. Typical clinical presentation of IgG4-RKD includes altered renal function with inconstant or no radiologic findings. Conversely, in the case we presented, a single nodule was detected upon imaging evaluation, thus mimicking malignancy. This raises the issue of a proper differential diagnosis. A multidisciplinary approach can be useful, although in clinical practice the selection of patients suspected of having IgG4-RKD is critical in the cases presenting with a renal mass that mimics malignancy.http://dx.doi.org/10.1155/2017/9690218
spellingShingle Daniele Bianchi
Luca Topazio
Gabriele Gaziev
Valerio Iacovelli
Pierluigi Bove
Alessandro Mauriello
Enrico Finazzi Agrò
IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass
Case Reports in Surgery
title IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass
title_full IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass
title_fullStr IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass
title_full_unstemmed IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass
title_short IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass
title_sort igg4 related kidney disease report of a case presenting as a renal mass
url http://dx.doi.org/10.1155/2017/9690218
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