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Adv Chronic Kidney Dis. 2017 Mar;24(2):94-100. doi: 10.1053/j.ackd.2016.12.001.

IgG4-Related Tubulointerstitial Nephritis.

Author information

1
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
2
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN. Electronic address: Cornell.Lynn@mayo.edu.

Abstract

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fibroinflammatory disorder that can involve nearly any organ. The disorder has increasingly become known as a distinct clinical entity during the last decade. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-RD in the kidney. Many patients with IgG4-TIN are diagnosed after IgG4-RD has been recognized in other organ systems, but the kidney may also be the first or only site involved. The presenting clinical features of IgG4-TIN are most commonly kidney insufficiency, kidney mass lesion(s), or both. On biopsy, IgG4-TIN shows a dense lymphoplasmacytic infiltrate, increased IgG4+ plasma cells, storiform fibrosis, and often tubular basement membrane immune complex deposits. Elevation of serum IgG4 often accompanies IgG4-RD; however, it is not specific in reaching the diagnosis. Like IgG4-RD in other organs, IgG4-TIN characteristically responds promptly to steroids, although there is a high relapse rate on discontinuation of immunosuppression. The pathogenesis of IgG4-RD is not understood.

KEYWORDS:

IgG4-related disease; Immune complex; Interstitial nephritis; Plasma cell

PMID:
28284385
DOI:
10.1053/j.ackd.2016.12.001
[Indexed for MEDLINE]

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