Goodpasture's disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques.
Goodpasture's disease is characterized by rapidly progressive glomerulonephritis, often accompanied by pulmonary hemorrhage, in association with deposition of antibodies in a linear pattern on the glomerular basement membrane (GBM). The diagnosis of Goodpasture's disease in patients with acute renal failure often relies on the use of immunoassays to detect circulating anti-GBM antibodies in serum samples. We describe three cases of Goodpasture's disease in which no circulating anti-GBM antibodies were detectable in serum by well-established enzyme-linked immunosorbent assay or Western blotting techniques. The diagnosis of Goodpasture's disease was confirmed by renal biopsy, with linear deposition of immunoglobulin along the GBM and crescentic glomerulonephritis. In addition, an alternative method of antibody detection using a highly sensitive biosensor system confirmed that circulating antibodies were present in sera from both patients tested. Because this technique is not routinely available for the detection of anti-GBM antibodies, we suggest that diagnosis always be confirmed with a renal biopsy, and despite negative serological test results using immunoassay, the diagnosis of Goodpasture's disease should still be considered in the correct clinical context.
Salama
Alan D
AD
Renal Section, Faculty of Medicine, Imperial College, Hammersmith Hospital, London, UK. asalama@rics.bwh.harvard.edu
Dougan
Tammy
T
Levy
Jeremy B
JB
Cook
H Terry
HT
Morgan
Steve H
SH
Naudeer
Sarah
S
Maidment
Geoff
G
George
Andrew J T
AJ
Evans
David
D
Lightstone
Liz
L
Pusey
Charles D
CD
D000293:Adolescent; D000328:Adult; D000368:Aged; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis* / Q000276:immunology; D001323:Autoantibodies / Q000097:blood*; D001485:Basement Membrane / Q000276:immunology; D001706:Biopsy; D015374:Biosensing Techniques; D015153:Blotting, Western; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007678:Kidney Glomerulus / Q000276:immunology*; D008297:Male
D001323:Autoantibodies; D007074:Immunoglobulin G