IgG4-related tubulointerstitial nephritis with membranous nephropathy

Fernando C. Fervenza, Gregory Downer, Laurence H. Beck, Sanjeev Sethi

Research output: Contribution to journalArticlepeer-review

34 Scopus citations


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
Issue number2
StatePublished - Aug 1 2011


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

ASJC Scopus subject areas

  • Nephrology


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