title: [IgG4-related disease and renal and urological involvement].
abstract: Hyper-IgG4 syndrome, or IgG4-related disease, is an emerging disorder, involving one or more organ(s), and characterized by "storiform" fibrosis and inflammatory lesions with a predominance of IgG4+ plasma cells and increased IgG4 serum levels. Since the first report of auto-immune pancreatitis, numerous organ lesions have been reported and have been found to occur in a same patient including: sialadenitis, dacryoadenitis, lymphadenopathy, liver and biliary tract involvement, and renal and retroperitoneal lesions. Renal involvement was first described in 2004 and usually presents as functional and/or morphological abnormalities. In most cases, renal pathological analysis reveals tubulointerstitial nephritis that is rarely associated with glomerular lesions. Retroperitoneal fibrosis is also a typical feature that may be associated with periaortitis or inflammatory abdominal aortic aneurysm. First line treatment is based on corticosteroid therapy. Short-term outcome is usually favorable. However, patients should be carefully monitored for relapses and long-term complications. Although the multiple organ lesions share common clinical, biological, radiological and pathological features, no consensus diagnostic criteria have yet been validated for IgG4-related disease. Ruling out differential diagnoses is thus mandatory. Our literature review provides nephrologists, urologists and pathologists with key elements that will help in the early diagnosis and proper management of this new and emerging disorder.
authors.affiliation: Service de néphrologie et dialyses, hôpital Tenon, Assistance publique des Hôpitaux de Paris, 4, rue de la Chine, 75020 Paris, France. mohamad.zaidan@hotmail.fr
authors.forename: Mohamad
authors.initials: M
authors.lastname: Zaidan
authors.forename: Mikael
authors.initials: M
authors.lastname: Ebbo
authors.forename: Isabelle
authors.initials: I
authors.lastname: Brochériou
authors.forename: Pierre
authors.initials: P
authors.lastname: Ronco
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Boffa
mesh_terms: D001014:Aortic Aneurysm / Q000473:pathology; D001025:Aortitis / Q000276:immunology / Q000473:pathology; D015433:Glomerulonephritis, Membranous / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007668:Kidney / Q000473:pathology; D009395:Nephritis, Interstitial / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D012185:Retroperitoneal Fibrosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G