An unusual case of anti-glomerular basement membrane disease presenting with nephrotic syndrome

Abstract

Anti-glomerular basement membrane (anti-GBM) disease is a vasculitic disease characterized by acute kidney injury, oliguria, hematuria and proteinuria. Proteinuria is rarely in the nephrotic range. A case of anti-GBM disease with proteinuria of 22.5 g/day is discussed. Immunofluorescence showed strong linear IgG deposits while electron microscopy showed widespread visceral epithelial cell foot cell process effacement. No electron dense immune complex-type deposits were identified. Pathology findings were not suggestive of simultaneous presentation of anti-GBM disease and other diseases associated with nephrotic range proteinuria. Anti-GBM disease should be considered in a comprehensive differential diagnosis of severe proteinuria.

Crucial Role of Mesangial Cell-derived Connective Tissue Growth Factor in a Mouse Model of Anti-Glomerular Basement Membrane Glomerulonephritis

“…We demonstrated the reduction of urinary protein, crescentic formation, mesangial expansion and electron dense deposits without changing heterologous, autologous antibodies or C3 complement deposition. The previous reports showed that discrepancy between IgG deposition and electron dense deposits in anti-GBM nephritis was documented 25 26 , which were consistent with our results.…”

Proliferative glomerulonephritis with linear immunoglobulin deposition: is this atypical antiglomerular basement membrane disease?

“…Although we had a good response to this therapy and are confident that it was key to inducing remission in our case, we cannot completely exclude that this response reflected a combined effect of both cyclophosphamide and rituximab. Our patient had severe proteinuria suggesting that diffuse podocyte injury was present and, despite not being able to confirm this hypothesis on our case because we did not have electronic microscopy available, some authors report that effacement of foot process is present in a significant number of anti-GBM disease cases 14. Rituximab may interfere directly with the podocytes, via direct binding with SMPDL-3b, protecting the structure and function of podocytes15; this may also be a mechanism by which rituximab was effective in our case.…”

Management of Double-Seropositive Anti-Glomerular Basement Membrane and Anti-Neutrophil Cytoplasmic Antibodies with 100% Crescentic Glomerulonephritis and Nephrotic Range Proteinuria in a Young Female

“…While our patient clearly does not fit into the category of atypical anti-GBM and the renal biopsy did not show any evidence to suggest membranous nephropathy, it could be proposed that, in a similar fashion, her proteinuria resulted from the degree of podocyte injury shown on electron microscopy [22,[24][25][26][27]. Her baseline health and high fitness level may have allowed her to maintain daily function for longer after the advent of anti-GBM antibodies, leading to a presentation with higher degree of podocyte and glomerular capillary wall injury and mimicking more chronic or indolent atypical disease courses.…”

“…Though rare, a few other case reports have described nephrotic range proteinuria in the setting of anti-GBM disease. Still, with negative testing for membranous nephropathy, focal segmental glomerulonephritis, and no history of NSAID use, the explanation for such a degree of proteinuria remains unclear [22,[24][25][26][27].…”