Introduction: IgG4-related disease (IgG4-RD) is a recent 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.
Aim: The aim of the present study was to review urologic involvement in IgG4-RD with the purpose of providing urologists with the proper background necessary for a preliminary assessment of possible urologic localization of this recent clinical entity. Indeed, patients are typically referred for immunologic management, often right after a differential diagnosis of urologic disease.
Materials and methods: A systematic search of PubMed® for both original and review articles published up until October 2015 was performed using keywords relating to IgG4 and to single specific urologic organs, structures, or anatomic sites. The search was then extended to Google® using the same search criteria in order to identify articles not indexed in PubMed®.
Results: IgG4-RD is a systemic condition potentially involving every urologic site. It can mimic malignancies and is often misdiagnosed due to its rarity.
Conclusions: A multidisciplinary approach to IgG4-RD should be required because it occasionally mimics other urologic diseases, including malignancies. Therefore, urologists should perform preliminary assessments to avoid inappropriate urologic treatments.
Keywords: Autoimmune pancreatitis; IgG4-related disease; Membranous glomerulonephritis; Pseudotumors; Tubulointerstitial nephritis; Urology.