title: A masquerading mass: an unusual presentation of IgG4-related systemic disease with tubulointerstitial nephritis.
abstract: IgG4 tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4 renal disease. When IgG4-TIN is accompanied by other systemic manifestations the disease is known as IgG4-related systemic disease (IgG4-RSD). IgG4-RSD is well recognised in the form of tubulointerstitial nephritis (IgG4-related TIN) and may present with renal failure by mimicking neoplasms (tumefactive lesions) or with both features. We describe a case of IgG4-RSD initially presenting as a submandibular mass and subsequently presenting two years later with secondary infiltration of the kidney causing tubulointerstitial nephritis. This case highlights the importance of recognising IgG4-RSD as a non-malignant disease with presentations having commonly shared features including tumour-like swelling of involved organs and its ability to mimic many systemic diseases. In the majority of patients it can be treated successfully with corticosteroids.
authors.affiliation: J Harty Daisy Hill Hospital Renal Unit 5 Hospital Road Newry BT35 8DR County Down Northern Ireland. Email john.harty@southerntrust.hscni.net.
authors.forename: B
authors.initials: B
authors.lastname: Quinn
authors.forename: J
authors.initials: J
authors.lastname: Harty
authors.forename: W
authors.initials: W
authors.lastname: Habeichi
mesh_terms: D003404:Creatinine / Q000097:blood; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D012793:Sialadenitis / Q000175:diagnosis* / Q000209:etiology
chemical_list: D007074:Immunoglobulin G; D003404:Creatinine
keywords: IgG4-related systemic disease (IgG4-RSD); Küttner’s tumour; chronic sclerosing sialadenitis; tubulointerstitial nephritis