Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
6161271 | Kidney International | 2013 | 8 Pages |
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 8.3Â g/day), and most had elevated serum creatinine (mean 2.2Â 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 1.2Â g/day), and most showed decreased serum creatinine (mean 1.4Â 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.
Keywords
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Authors
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,