title: [IgG4-related kidney disease. Diagnosis and treatment].
abstract: IgG4-related disease (IgG4-RD) is a systemic inflammatory disorder that can affect most organs/tissues like sarcoidosis. The kidney is one of the most frequently affected organs. While tubulointerstitial nephritis (TIN) with characteristic imaging findings is the representative lesion of IgG4-related kidney disease (IgG4-RKD), a variety of glomerular lesions, particularly membranous nephropathy, sometimes overlap on TIN. Clinically, either decreased renal function and/or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography are typical presenting features. Histologically, plasma cell (PC)-rich TIN accompanied by characteristic fibrosis called storiform fibrosis with dense IgG4-positive PC infiltration is a typical finding. Although a swift response to corticosteroid is a very important feature of IgG4-RKD, in cases with moderately to severely decreased renal function before therapy, only partial recovery of renal function is obtained. This review provides a comprehensive overview of IgG4-RKD from the clinical, laboratory, imaging, and histological aspects and also addresses some of the therapeutic issues concerning it.
authors.affiliation: Division of Rheumatology, Kanazawa University Hospital.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kawano
authors.forename: Ichiro
authors.initials: I
authors.lastname: Mizushima
authors.forename: Kazunori
authors.initials: K
authors.lastname: Yamada
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Taniguchi
authors.forename: Takako
authors.initials: T
authors.lastname: Saeki
mesh_terms: D000368:Aged; D005260:Female; D005355:Fibrosis; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007668:Kidney / Q000473:pathology; D007674:Kidney Diseases / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy*; D008297:Male; D009395:Nephritis, Interstitial / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage; D011379:Prognosis; D014057:Tomography, X-Ray Computed
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D011239:Prednisolone