title: Clinicopathological findings of immunoglobulin G4-related kidney disease.
abstract: Immunoglobulin (Ig) G4-related kidney disease characterizing tubulointerstitial nephritis (TIN) is an organ complication recognized in IgG4-related systemic diseases that has some unique aspects compared to other types of TIN. TIN lesions in the kidney can be tumor-like, focal or diffuse. Abnormal urinalysis is usually mild or absent even in the cases with deteriorated renal dysfunction. Some cases are accidentally diagnosed from radiological findings without renal dysfunction and/or abnormal urinalysis. The typical pathological findings of TIN are unique fibrosis and infiltration of massive lymphocytes and IgG4-positive plasma cells. Glomerular lesions are rare but the complication of mesangial proliferative glomerulonephritis and membranous nephropathy is occasionally reported. Pathogenic mechanisms are unclear until now; however, auto-immune and allergic mechanisms have been suspected from laboratory data. The initial response to steroid agents is generally favorable; however, recurrence is possible after the discontinuation of steroid treatment. Long-term follow-up is necessary with continuous systemic checks for organ disorders due to IgG4-related systemic diseases.
authors.affiliation: Division of Nephrology and Kidney Center, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo 650-0017, Japan. snishi@med.kobe-u.ac.jp
authors.forename: Shinichi
authors.initials: S
authors.lastname: Nishi
authors.forename: Naofumi
authors.initials: N
authors.lastname: Imai
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Yoshida
authors.forename: Yumi
authors.initials: Y
authors.lastname: Ito
authors.forename: Takako
authors.initials: T
authors.lastname: Saeki
mesh_terms: D000818:Animals; D005355:Fibrosis; D015433:Glomerulonephritis, Membranous / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D007668:Kidney / Q000187:drug effects / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D008214:Lymphocytes / Q000276:immunology; D009395:Nephritis, Interstitial / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D010950:Plasma Cells / Q000276:immunology*; D011237:Predictive Value of Tests; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D007074:Immunoglobulin G; D013256:Steroids