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Semin Liver Dis 2016; 36(03): 283-290
DOI: 10.1055/s-0036-1584316
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis

Mitsuhiro Kawano
1   Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan
,
Kazunori Yamada
1   Division of Rheumatology, Department of Internal Medicine, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan
› Author Affiliations
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Publication History

Publication Date:
28 July 2016 (online)

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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.

Keywords

immunoglobulin G4-related kidney disease - tubulointerstitial nephritis - membranous glomerulonephritis - retroperitoneal fibrosis - hydronephrosis
 

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