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Renal injury due to anti-glomerular basement membrane antibody-mediated glomerulonephritis without circulating antibody.

Schena FP, Dash A, Fatima H, Grewal M, Galphin C, Paueksakon P - Clin Kidney J (2012)

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Microbiology and Immunology , Vanderbilt University Medical Center , Nashville, TN , USA.

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Patients with anti-glomerular basement membrane (GBM) antibody-mediated GN usually present with rapidly progressive glomerulonephritis (GN)... The disease is caused by antibodies to the noncollagenous-1 (NC1) domain of the α3 chain of collagen IV, termed Goodpasture's antigen, in the majority of cases... The specificity of the antibody can be confirmed by western blotting... False-negative serology antibody results may occur, generally in patients with isolated mild pulmonary disease... False-positive results can occur in certain assays when circulating antibodies are generated to GBM antigens other than the NC1 domain of the α3 chain of collagen IV or in patients with polyclonal immune stimulation... The renal biopsy showed necrotizing crescentic GN superimposed on idiopathic nodular glomerulosclerosis (ING) and linear staining of IgG along the glomerular capillary walls... Circulating anti-GBM antibodies were found to be absent by standard immunoassays... Nodular sclerosis may be present in diabetic nephropathy and have linear GBM accentuation... However, our patient did not have diabetes and there was no history of glucose intolerance, and electron microscopy showed normal GBM thickness, thus not indicative of diabetic nephropathy... Rarely, anti-GBM disease occurs in combination with other diseases... The most common concurrent process includes ANCA-associated crescentic GN and membranous glomerulopathy... These are directed against Goodpasture's antigen (NC1 domain of the α3 chain of collagen IV)... We report a case of anti-GBM antibody-mediated GN superimposed on ING in the absence of circulating anti-GBM antibodies by ELISA... This false negative result occurs only in 2–3% of patients... In this reported case, the renal biopsy was the only test detecting anti-GBM antibody-mediated GN.

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Related in: MedlinePlus

A glomerulus with nodular sclerosis and segmental fibrinoid necrosis (arrow) (Jones' silver stain, ×400).
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SFS106F1: A glomerulus with nodular sclerosis and segmental fibrinoid necrosis (arrow) (Jones' silver stain, ×400).

Mentions: The renal biopsy specimen included two pieces of cortex containing 17 glomeruli, 4 of which were globally sclerosed. Six glomeruli showed crescents, one cellular and five fibrocellular, and three glomeruli had focal fibrinoid necrosis (Figure 1) and one also had segmental endocapillary proliferation. The GBM revealed segmental double contours but there were no spikes, holes or corrugation. In addition, there was a moderate increase in mesangial matrix and mild increase in cells with focal nodular expansion with mesangiolysis and microaneurysmal dilatation of the capillaries with fragmented red blood cells within the mesangial nodules (Figure 2). The mesangial nodules stained positive with Jones' silver stain and periodic acid Schiff stain. There was ∼30% patchy, well-delineated interstitial fibrosis with proportional tubular atrophy and moderate lymphoplasmacytic infiltrate. There were no features suggestive of obstruction such as dilated Bowman's spaces or tubular dilatation. There were no fractured casts, crystals or polarizable materials. Arterioles showed moderate hyalinosis, but not demonstrated to involve both afferent and efferent arterioles. Arteries showed moderate intimal fibrosis without fibrinoid necrosis or vasculitis.Fig. 1.


Renal injury due to anti-glomerular basement membrane antibody-mediated glomerulonephritis without circulating antibody.

Schena FP, Dash A, Fatima H, Grewal M, Galphin C, Paueksakon P - Clin Kidney J (2012)

A glomerulus with nodular sclerosis and segmental fibrinoid necrosis (arrow) (Jones' silver stain, ×400).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400546&req=5

SFS106F1: A glomerulus with nodular sclerosis and segmental fibrinoid necrosis (arrow) (Jones' silver stain, ×400).
Mentions: The renal biopsy specimen included two pieces of cortex containing 17 glomeruli, 4 of which were globally sclerosed. Six glomeruli showed crescents, one cellular and five fibrocellular, and three glomeruli had focal fibrinoid necrosis (Figure 1) and one also had segmental endocapillary proliferation. The GBM revealed segmental double contours but there were no spikes, holes or corrugation. In addition, there was a moderate increase in mesangial matrix and mild increase in cells with focal nodular expansion with mesangiolysis and microaneurysmal dilatation of the capillaries with fragmented red blood cells within the mesangial nodules (Figure 2). The mesangial nodules stained positive with Jones' silver stain and periodic acid Schiff stain. There was ∼30% patchy, well-delineated interstitial fibrosis with proportional tubular atrophy and moderate lymphoplasmacytic infiltrate. There were no features suggestive of obstruction such as dilated Bowman's spaces or tubular dilatation. There were no fractured casts, crystals or polarizable materials. Arterioles showed moderate hyalinosis, but not demonstrated to involve both afferent and efferent arterioles. Arteries showed moderate intimal fibrosis without fibrinoid necrosis or vasculitis.Fig. 1.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Microbiology and Immunology , Vanderbilt University Medical Center , Nashville, TN , USA.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Patients with anti-glomerular basement membrane (GBM) antibody-mediated GN usually present with rapidly progressive glomerulonephritis (GN)... The disease is caused by antibodies to the noncollagenous-1 (NC1) domain of the α3 chain of collagen IV, termed Goodpasture's antigen, in the majority of cases... The specificity of the antibody can be confirmed by western blotting... False-negative serology antibody results may occur, generally in patients with isolated mild pulmonary disease... False-positive results can occur in certain assays when circulating antibodies are generated to GBM antigens other than the NC1 domain of the α3 chain of collagen IV or in patients with polyclonal immune stimulation... The renal biopsy showed necrotizing crescentic GN superimposed on idiopathic nodular glomerulosclerosis (ING) and linear staining of IgG along the glomerular capillary walls... Circulating anti-GBM antibodies were found to be absent by standard immunoassays... Nodular sclerosis may be present in diabetic nephropathy and have linear GBM accentuation... However, our patient did not have diabetes and there was no history of glucose intolerance, and electron microscopy showed normal GBM thickness, thus not indicative of diabetic nephropathy... Rarely, anti-GBM disease occurs in combination with other diseases... The most common concurrent process includes ANCA-associated crescentic GN and membranous glomerulopathy... These are directed against Goodpasture's antigen (NC1 domain of the α3 chain of collagen IV)... We report a case of anti-GBM antibody-mediated GN superimposed on ING in the absence of circulating anti-GBM antibodies by ELISA... This false negative result occurs only in 2–3% of patients... In this reported case, the renal biopsy was the only test detecting anti-GBM antibody-mediated GN.

No MeSH data available.


Related in: MedlinePlus