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Case Reports
. 2010 Aug;3(4):357-9.
doi: 10.1093/ndtplus/sfq076. Epub 2010 May 2.

Steroid-responsive nephrotic syndrome in a patient with proliferative glomerulonephritis with monoclonal IgG deposits with pure mesangial proliferative features

Atsushi Komatsuda  1 , Hideki Wakui  1 , Hiroshi Ohtani  2 , Takashi Nimura  3 , Ken-Ichi Sawada  1
Affiliations
Case Reports

Steroid-responsive nephrotic syndrome in a patient with proliferative glomerulonephritis with monoclonal IgG deposits with pure mesangial proliferative features

Atsushi Komatsuda et al. NDT Plus. 2010 Aug.

Abstract

A 78-year-old woman developed acute-onset nephrotic syndrome. A renal biopsy showed mild mesangial proliferative glomerulonephritis. Immunofluorescence studies revealed granular IgG3- λ deposits within the mesangial area and along the glomerular capillary walls. Electron microscopy showed mesangial and subendothelial granular electron-dense deposits. The pattern of deposition was predominantly mesangial. Serum or urine monoclonal proteins were not detected. Middle-dose steroid therapy induced a rapid remission of nephrotic syndrome. We consider that this is the first case of steroid-responsive nephrotic syndrome due to an extremely rare glomerular disease, proliferative glomerulonephritis with monoclonal IgG deposits associated with pure mesangial proliferative features.

Keywords: proliferative glomerulonephritis with monoclonal IgG deposits; steroid-responsive nephrotic syndrome.

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Figures

Fig. 1
Fig. 1
Light microscopy shows a mild mesangial proliferation (arrow). Periodic acid–Schiff staining (×400).
Fig. 2
Fig. 2
Immunofluorescence microscopy for IgG-heavy chain (A), γ3-heavy chain (B), κ-light chain (C) and λ-light chain (D) displays a strong staining for IgG-heavy chain, γ3-heavy chain and λ-light chain within the mesangial area and along the glomerular capillary walls with a granular texture, but no significant staining for κ-light chain (×400).
Fig. 3
Fig. 3
Electron microscopy reveals granular electron-dense deposits in mesangial and subendothelial location (arrows) and podocyte foot process effacement (arrowhead). The bar represents 1 μm.
See this image and copyright information in PMC

References

    1. Santostefano M, Zanchelli F, Zaccaria A, et al. The ultrastructural basis of renal pathology in monoclonal gammopathies. J Nephrol. 2005;18:659–675. - PubMed
    1. Preud’homme J-L, Aucouturier P, Touchard G, et al. Monoclonal immunoglobulin deposition disease (Randall type). Relationship with structural abnormalities of immunoglobulin chains. Kidney Int. 1994;46:965–972. - PubMed
    1. Nasr SH, Markowitz GS, Stokes MB, et al. Proliferative glomerulonephritis with monoclonal IgG deposits: a distinct entity mimicking immune-complex glomerulonephritis. Kidney Int. 2004;65:85–96. - PubMed
    1. Komatsuda A, Masai R, Ohtani H, et al. Monoclonal immunoglobulin deposition disease associated with membranous features. Nephrol Dial Transplant. 2008;23:3888–3894. - PubMed
    1. Masai R, Wakui H, Komatsuda A, et al. Characteristics of proliferative glomerulonephritis with monoclonal IgG deposits associated with membranoproliferative features. Clin Nephrol. 2009;72:46–54. - PubMed

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