IgG4-Related Tubulointerstitial Nephritis

Pingchuan Zhang, Lynn D. Cornell

Research output: Contribution to journalReview article

7 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)94-100
Number of pages7
JournalAdvances in Chronic Kidney Disease
Volume24
Issue number2
DOIs
StatePublished - Mar 1 2017

Keywords

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

ASJC Scopus subject areas

  • Nephrology

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