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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.

No MeSH data available.


Related in: MedlinePlus

Electron microscopy shows normal thickness of lamina densa with a diffuse increase in lamina rara interna (arrow) and extensive foot process effacement (transmission electron microscopy, ×5200).
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SFS106F4: Electron microscopy shows normal thickness of lamina densa with a diffuse increase in lamina rara interna (arrow) and extensive foot process effacement (transmission electron microscopy, ×5200).

Mentions: By electron microscopy, GBM showed normal thickness in areas away from increase in lamina rara interna with an average of 450 nm (average GBM thickness for adult man in our laboratory 370 ± 42 nm) (Figure 4). There was a diffuse increase in lamina rara interna. There were no immune complex deposits. There were no fibrin tactoids or reticular arrays. There was a moderate increase in mesangial matrix. The foot processes were ∼90% effaced. There were no TBM deposits.Fig. 4.


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)

Electron microscopy shows normal thickness of lamina densa with a diffuse increase in lamina rara interna (arrow) and extensive foot process effacement (transmission electron microscopy, ×5200).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

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

SFS106F4: Electron microscopy shows normal thickness of lamina densa with a diffuse increase in lamina rara interna (arrow) and extensive foot process effacement (transmission electron microscopy, ×5200).
Mentions: By electron microscopy, GBM showed normal thickness in areas away from increase in lamina rara interna with an average of 450 nm (average GBM thickness for adult man in our laboratory 370 ± 42 nm) (Figure 4). There was a diffuse increase in lamina rara interna. There were no immune complex deposits. There were no fibrin tactoids or reticular arrays. There was a moderate increase in mesangial matrix. The foot processes were ∼90% effaced. There were no TBM deposits.Fig. 4.

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