Membranous glomerulonephritis is a manifestation of IgG4-related disease

Mariam P. Alexander, Christopher P. Larsen, Ian W. Gibson, Samih H. Nasr, Sanjeev Sethi, Mary E. Fidler, Yassaman Raissian, Naoki Takahashi, Suresh Chari, Thomas C. Smyrk, Lynn D. Cornell

Research output: Contribution to journalArticle

101 Scopus citations

Abstract

IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that typically manifests as fibro-inflammatory masses that can affect nearly any organ system. Renal involvement by IgG4-RD usually takes the form of IgG4-related tubulointerstitial nephritis, but cases of membranous glomerulonephritis (MGN) have also been described. Here we present a series of 9 patients (mean age at diagnosis 58 years) with MGN associated with IgG4-RD. All patients showed MGN on biopsy, presented with proteinuria (mean 8.3 g/day), and most had elevated serum creatinine (mean 2.2 mg/dl). Seven patients had known extrarenal involvement by IgG4-RD, with 5 patients having concurrent IgG4-related tubulointerstitial nephritis. Immunohistochemical analysis for the phospholipase A2 receptor, a marker of primary MGN, was negative in all 8 biopsies so examined. Six of 7 patients with available follow-up (mean 39 months) were treated with immunosuppressive agents; one untreated patient developed end-stage renal disease and underwent transplantation, without recurrence at 12 years after transplant. All 6 treated patients showed decreased proteinuria (mean 1.2 g/day), and most showed decreased serum creatinine (mean 1.4 mg/dl). Thus, MGN should be included in the spectrum of IgG4-RD and should be suspected in proteinuric IgG4-RD patients. Conversely, patients with MGN and an appropriate clinical history should be evaluated for IgG4-RD.

Original languageEnglish (US)
Pages (from-to)455-462
Number of pages8
JournalKidney international
Volume83
Issue number3
DOIs
StatePublished - Mar 2013

Keywords

  • IgG4-related sclerosing disease
  • autoimmune pancreatitis
  • interstitial nephritis
  • membranous glomerulopathy
  • membranous nephropathy

ASJC Scopus subject areas

  • Nephrology

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    Alexander, M. P., Larsen, C. P., Gibson, I. W., Nasr, S. H., Sethi, S., Fidler, M. E., Raissian, Y., Takahashi, N., Chari, S., Smyrk, T. C., & Cornell, L. D. (2013). Membranous glomerulonephritis is a manifestation of IgG4-related disease. Kidney international, 83(3), 455-462. https://doi.org/10.1038/ki.2012.382