title: IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis.
abstract: Immunoglobulin G4-related kidney disease (IgG4-RKD) is the collective name encompassing renal parenchymal and renal pelvic lesions. The hallmark of renal parenchymal lesions of IgG4-related disease is plasma cell-rich tubulointerstitial nephritis with numerous IgG4-positive plasma cells and characteristic fibrosis. In addition, glomerular lesions are sometimes present, with membranous glomerulonephritis being the most common. Although IgG4-RKD shows good responsiveness to corticosteroid therapy, follow-up imaging studies have revealed that partial cortical scars persist when the start of therapy is delayed. In this review, the authors provide an overview of the latest knowledge of IgG4-RKD, focusing in particular on its pathological and imaging characteristic features.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kawano
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
authors.forename: Kazunori
authors.initials: K
authors.lastname: Yamada
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007668:Kidney / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D009395:Nephritis, Interstitial / Q000175:diagnosis / Q000473:pathology*; D049268:Positron-Emission Tomography; D012185:Retroperitoneal Fibrosis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G