IgG4-related tubulointerstitial nephritis with membranous nephropathy

Fernando Custodio Fervenza, Gregory Downer, Laurence H. Beck, Sanjeev M Sethi

Research output: Contribution to journalArticle

33 Citations (Scopus)

Abstract

We describe a 67-year-old woman who presented with significant proteinuria and hematuria. Kidney biopsy showed immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis (TIN) with concurrent membranous nephropathy. IgG4-related TIN is a recently described entity that presents with progressive decreased kidney function and is characterized by a plasma cellrich infiltrate that is positive for IgG4. It is associated with patchy, often well-localized, tubular atrophy and interstitial fibrosis. Workup for circulating antiphospholipase A2 receptor antibodies was negative, suggesting that the membranous nephropathy was not "primary" and may be linked to the IgG4-related disease. The presence of significant proteinuria and hematuria in the setting of IgG4-related TIN should raise suspicion of a glomerular disease. It is important to correctly diagnose IgG4-related TIN and concurrent membranous nephropathy because the lesion responds well to steroid therapy.

Original languageEnglish (US)
Pages (from-to)320-324
Number of pages5
JournalAmerican Journal of Kidney Diseases
Volume58
Issue number2
DOIs
StatePublished - Aug 2011

Fingerprint

Interstitial Nephritis
Membranous Glomerulonephritis
Immunoglobulins
Hematuria
Proteinuria
Kidney
varespladib methyl
Atrophy
Fibrosis
Steroids
Biopsy
Antibodies

Keywords

  • immunoglobulin G4 (IgG4)-related sclerosing disease
  • Immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis (TIN)
  • membranous nephropathy

ASJC Scopus subject areas

  • Nephrology

Cite this

IgG4-related tubulointerstitial nephritis with membranous nephropathy. / Fervenza, Fernando Custodio; Downer, Gregory; Beck, Laurence H.; Sethi, Sanjeev M.

In: American Journal of Kidney Diseases, Vol. 58, No. 2, 08.2011, p. 320-324.

Research output: Contribution to journalArticle

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