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Circulating Anti-Glomerular Basement Membrane Antibodies With Predominance of Subclass IgG4 and False-Negative Immunoassay Test Results in Anti-Glomerular Basement Membrane Disease
Skåne University Hospital, Sweden .
Sahlgrens University Hospital, Sweden .
Karolinska University Hospital, Sweden .
Skåne University Hospital, Sweden .
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2014 (English)In: American Journal of Kidney Diseases, ISSN 0272-6386, E-ISSN 1523-6838, Vol. 63, no 2, 289-293 p.Article in journal (Refereed) Published
Abstract [en]

Autoantibodies against a constituent of the glomerular basement membrane (GBM), the alpha 3-chain of type IV collagen, can cause both rapidly progressive glomerulonephritis and alveolar hemorrhage, referred to as anti-GBM disease or Goodpasture disease. Anti-GBM antibodies generally are of immunoglobulin G subclass 1 (IgG1) and can in most cases readily be detected in the circulation using enzyme-linked immunosorbent assays (ELISAs). We report 4 cases in which anti-GBM ELISA yielded negative or borderline results despite life-threatening disease. All 4 patients had positive results by IgG4 anti-GBM ELISA and all had undetectable anti-neutrophil cytoplasmic antibody. All cases were confirmed with kidney biopsy. Two of the patients showed higher signal in anti-GBM ELISA when using a nondenaturing coating buffer. All 4 were young women with severe alveolar hemorrhage and favorable renal outcome, suggesting that patients with predominance of IgG4 autoantibodies may constitute a distinct subgroup of anti-GBM disease. We conclude that patients with idiopathic alveolar hemorrhage can have anti-GBM disease detected by only IgG subclass-specific tests or kidney biopsy.

Place, publisher, year, edition, pages
Elsevier , 2014. Vol. 63, no 2, 289-293 p.
Keyword [en]
Anti-glomerular basement membrane (anti-GBM); Goodpasture disease; immunoglobulin G4 (IgG4)
National Category
Medical and Health Sciences
URN: urn:nbn:se:liu:diva-104282DOI: 10.1053/j.ajkd.2013.08.032ISI: 000329980100018OAI: diva2:697201
Available from: 2014-02-17 Created: 2014-02-14 Last updated: 2014-03-12

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Segelmark, Mårten
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Division of Drug ResearchFaculty of Health SciencesDepartment of Nephrology
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