Circulating Anti-Glomerular Basement Membrane Antibodies With Predominance of Subclass IgG4 and False-Negative Immunoassay Test Results in Anti-Glomerular Basement Membrane Disease

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.

Anti-glomerular basement membrane (anti-GBM); Goodpasture disease; immunoglobulin G4 (IgG4)
MEDICINE
MEDICIN

Herlitz, HansSahlgrens University Hospital, Sweden (författare)
Lundberg, SigridKarolinska Institutet (författare)
Selga, DainaSkåne University Hospital, Sweden (författare)
Molne, JohanSahlgrens University Hospital, Sweden (författare)
Wieslander, JorgenEurodiagnost AB, Sweden (författare)
Segelmark, MårtenÖstergötlands Läns Landsting,Linköpings universitet,Avdelningen för läkemedelsforskning,Hälsouniversitetet,Njurmedicinska kliniken US(Swepub:liu)marse71 (författare)
Skåne University Hospital, SwedenSahlgrens University Hospital, Sweden (creator_code:org_t)

Ingår i:American Journal of Kidney Diseases: Elsevier63:2, s. 289-2930272-63861523-6838

Av författaren/redakt...: Ohlsson, Sophie; Herlitz, Hans; Lundberg, Sigrid; Selga, Daina; Molne, Johan; Wieslander, Jorg ...; visa fler...; Segelmark, Mårte ...; visa färre...

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