IgG4-Related Tubulointerstitial Nephritis

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

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.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G / immunology*
  • Magnetic Resonance Imaging
  • Nephritis, Interstitial / diagnosis*
  • Nephritis, Interstitial / drug therapy
  • Nephritis, Interstitial / immunology*
  • Nephritis, Interstitial / pathology
  • Tomography, X-Ray Computed

Substances

  • Immunoglobulin G