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Case Reports in Surgery
Volume 2017 (2017), Article ID 9690218, 6 pages
Case Report

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

1Department of Urology, Policlinico Tor Vergata, Rome, Italy
2Anatomic Pathology, Department of Experimental Medicine and Surgery, Tor Vergata University, Rome, Italy

Correspondence should be addressed to Daniele Bianchi

Received 16 April 2017; Revised 2 July 2017; Accepted 20 July 2017; Published 22 August 2017

Academic Editor: Marcus L. Quek

Copyright © 2017 Daniele Bianchi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.