Circulating anti-glomerular basement membrane antibodies with predominance of subclass IgG4 and false-negative immunoassay test results in anti-glomerular basement membrane disease

Research output: Contribution to journalArticle


Autoantibodies against a constituent of the glomerular basement membrane (GBM), the α3-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 antineutrophil 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.


External organisations
  • Sahlgrenska University Hospital
  • Eurodiagnostica AB
  • Skåne University Hospital
  • Karolinska University Hospital
  • Linköping University
  • Östergötland County Council
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Urology and Nephrology


  • Adolescent, Adult, Anti-Glomerular Basement Membrane Disease/blood, Autoantibodies/biosynthesis, False Negative Reactions, Female, Humans, Immunoassay/methods, Immunoglobulin G/biosynthesis, Young Adult
Original languageEnglish
Pages (from-to)289-93
Number of pages5
JournalAmerican Journal of Kidney Diseases
Issue number2
Publication statusPublished - 2014 Feb
Publication categoryResearch
Externally publishedYes