Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease.
A 68-year-old Caucasian woman presented to the hospital with nodular pulmonary infiltrates and acute renal failure. Wegener's granulomatosis was initially considered to be most likely because of the presence of increased serum levels of c-anti-neutrophil cytoplasmic antibodies (c-ANCA). A consultation through the Internet after a renal biopsy demonstrated crescentic, necrotizing glomerulonephritis and linear deposits of immunoglobulin G (IgG) and complement C3, typical of anti-glomerular basement membrane (GBM) disease. Hemodialysis was instituted; however, the patient suddenly developed a massive cerebral hemorrhage and died before full therapy could take effect. Postmortem analysis of the patient's sera revealed high titers of IgG against the alpha 3 NC1 domain of type IV collagen. Serologic evidence of both p-ANCA and anti-GBM antibodies are becoming more frequently recognized in the setting of rapidly progressive glomerulonephritis. The patient reported here had the unusual combination of c-ANCA antibodies with anti-GBM disease, and this association raises complex questions regarding the pathogenesis of this type of renal injury.
Kalluri
R
R
Penn Center for Molecular Studies of Kidney Diseases, University of Pennsylvania, Philadelphia, USA.
Meyers
K
K
Mogyorosi
A
A
Madaio
M P
MP
Neilson
E G
EG
D058186:Acute Kidney Injury / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D000368:Aged; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis / Q000276:immunology*; D000906:Antibodies / Q000032:analysis / Q000276:immunology*; D001323:Autoantibodies; D001344:Autopsy; D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000276:immunology*; D006801:Humans; D006435:Renal Dialysis
D000906:Antibodies; D001323:Autoantibodies; C051951:antiglomerular basement membrane antibody