Binding Truths: Atypical Anti−Glomerular Basement Membrane Disease Mediated by IgA Anti−Glomerular Basement Membrane Antibodies Targeting the α1 Chain of Type IV Collagen

Antonelou, Marilina; Henderson, Scott; Bhangal, Gurjeet; Heptinstall, Lauren; Oliveira, Ben; Hamour, Sally; Harber, Mark; Salama, Alan D.

doi:10.1016/j.ekir.2018.08.005

Cited by 15 publications

(21 citation statements)

References 19 publications

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“…Anti-GBM disease is a rare disease, with an incidence of one per million population/year in European populations (1). IgA mediated forms of this disease are even rarer, with only 13 cases reported in the literature (Table 1) (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15).…”

Section: Discussionmentioning

confidence: 99%

IgA mediated anti-glomerular basement membrane disease with associated circulating anti-neutrophil cytoplasm antibodies

et al. 2023

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Anti-glomerular basement membrane (anti-GBM) disease is an aggressive small vessel vasculitis usually mediated by IgG autoantibodies. We describe the case of a 73-year-old male with rapidly progressive renal failure that was diagnosed with IgA mediated anti-GBM disease associated with circulating anti-neutrophil cytoplasm antibodies (ANCA), where the diagnosis was established on kidney biopsy by detecting linear deposition of IgA along the GBM on immunofluorescence microscopy. Despite an intensive immunosuppressive regimen with plasmapheresis, steroids and oral cyclophosphamide, the disease progressed to end-stage renal failure and the patient was started on hemodialysis.

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Section: Discussionmentioning

confidence: 99%

IgA mediated anti-glomerular basement membrane disease with associated circulating anti-neutrophil cytoplasm antibodies

et al. 2023

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“…described a case of anti-GBM disease mediated by IgA anti-GBM antibody against collagenous domain of α1 Col (IV). [ 7 ] It is also possible that anti-GBM antibody in the blood may all become deposited in the kidney with time.…”

Section: Discussionmentioning

confidence: 99%

Recurrent, atypical anti-glomerular basement membrane disease

Jamboti¹,

Sinniah²,

D’Orsogna³

et al. 2021

Indian J Nephrol

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Anti-glomerular basement membrane disease (GBM) (Goodpasture's disease) typically presents with acute manifestations of rapidly progressive glomerulonephritis often accompanied by lung haemorrhage. Anti-GBM disease is usually monophasic. However, atypical presentations with indolent renal involvement are being increasingly recognized. Herein we report a patient who presented with lung haemorrhage, minimal renal involvement, and negative result for serum anti-GBM antibody, while immunofluorescence examination of the renal biopsy provided the diagnosis leading to the institution right treatment with excellent response. Interestingly, he had presented 10 years earlier with lung hemorrhage, more significant renal involvement clinically and histologically, with positive serum anti-GBM antibody. The present case is intended to increase our awareness regarding the variable presentations of anti-GBM disease, such as with negative serology and recurrence of anti-GBM disease. The presentation of anti-GBM nephritis with non-proliferative, non-crescentic glomerulonephritis is also highlighted. The possible explanations for negative serum anti-GBM antibody are explored with a brief review of literature.

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“…Finally, rare cases of IgA-anti GBM disease have been reported and these follow a rapidly progressive course, as for IgG-anti -GBM disease. The antigenic target of these molecules does not appear to be the classical 3(IV) NC1, but other (IV) chain molecules in the GBM [10].…”

Section: Pathogenesismentioning

confidence: 96%

IgA vasculitis and anti-GBM disease: two ends of a spectrum of immune complex vasculitis

Salama

2020

Rheumatology

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Two immune complex vasculitides, IgA vasculitis (IgAV) and anti-GBM disease, represent polar extremes with regard to our understanding of disease pathogenesis, standardized management protocols and outcomes. This report compares our current approach to these uncommon entities in adults. Both diseases demonstrate degrees of small vessel necrosis and glomerular crescent formation. IgAV has an antibody response directed against unknown antigens, is often treated conservatively and has poorly studied long term renal outcomes. By contrast, anti-GBM disease presents with rapidly progressive glomerulonephritis and often results in end stage renal failure, despite intensive immunosuppression. Rarely, some cases of anti-GBM disease may be IgA predominant and bind other α-chains present in the GBM, but their clinical course is as for other anti-GBM disease patients but not IgAV, suggesting that the antigenic target rather than the antibody subclass is the critical factor in determining disease outcome. However, both conditions are associated with increased mortality in adults and result in significant chronic kidney disease and hypertension.

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Binding Truths: Atypical Anti−Glomerular Basement Membrane Disease Mediated by IgA Anti−Glomerular Basement Membrane Antibodies Targeting the α1 Chain of Type IV Collagen

Cited by 15 publications

References 19 publications

IgA mediated anti-glomerular basement membrane disease with associated circulating anti-neutrophil cytoplasm antibodies

IgA mediated anti-glomerular basement membrane disease with associated circulating anti-neutrophil cytoplasm antibodies

Recurrent, atypical anti-glomerular basement membrane disease

IgA vasculitis and anti-GBM disease: two ends of a spectrum of immune complex vasculitis

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