Membranous glomerulonephritis is a manifestation of IgG4-related disease

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.

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 and Lynn D Cornell
Kidney Int 2013 83: 455-462; advance online publication, December 19, 2012;

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Last modified on Friday, 28 March 2014 11:19

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