Journal
AMERICAN JOURNAL OF KIDNEY DISEASES
Volume 63, Issue 2, Pages 289-293Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1053/j.ajkd.2013.08.032
Keywords
Anti-glomerular basement membrane (anti-GBM); Goodpasture disease; immunoglobulin G4 (IgG4)
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Funding
- Swedish Kidney Foundation
- Ingrid Asp Foundation
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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. (C) 2014 by the National Kidney Foundation, Inc.
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