Membranous glomerulonephritis is a manifestation of IgG4-related disease

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

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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

Fingerprint

Membranous Glomerulonephritis
Immunoglobulin G
Interstitial Nephritis
Proteinuria
Phospholipase A2 Receptors
Creatinine
Biopsy
Immune System Diseases
Immunosuppressive Agents
Serum
Chronic Kidney Failure
Transplantation
Transplants
Kidney
Recurrence

Keywords

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

ASJC Scopus subject areas

  • Nephrology

Cite this

Alexander, M. P., Larsen, C. P., Gibson, I. W., Nasr, S. H., Sethi, S. M., Fidler, M. E., ... 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

Membranous glomerulonephritis is a manifestation of IgG4-related disease. / Alexander, Mariam P; Larsen, Christopher P.; Gibson, Ian W.; Nasr, Samih H.; Sethi, Sanjeev M; Fidler, Mary E.; Raissian, Yassaman; Takahashi, Naoki; Chari, Suresh T; Smyrk, Thomas Christopher; Cornell, Lynn D.

In: Kidney International, Vol. 83, No. 3, 03.2013, p. 455-462.

Research output: Contribution to journalArticle

Alexander, MP, Larsen, CP, Gibson, IW, Nasr, SH, Sethi, SM, Fidler, ME, Raissian, Y, Takahashi, N, Chari, ST, Smyrk, TC & Cornell, LD 2013, 'Membranous glomerulonephritis is a manifestation of IgG4-related disease', Kidney International, vol. 83, no. 3, pp. 455-462. https://doi.org/10.1038/ki.2012.382
Alexander, Mariam P ; Larsen, Christopher P. ; Gibson, Ian W. ; Nasr, Samih H. ; Sethi, Sanjeev M ; Fidler, Mary E. ; Raissian, Yassaman ; Takahashi, Naoki ; Chari, Suresh T ; Smyrk, Thomas Christopher ; Cornell, Lynn D. / Membranous glomerulonephritis is a manifestation of IgG4-related disease. In: Kidney International. 2013 ; Vol. 83, No. 3. pp. 455-462.
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