Goodpasture’s syndrome is a rare disorder characterized by pulmonary hemorrhage, glomerulonephritis, and antiglomerular basement membrane antibodies.
Anti–glomerular basement membrane (anti-GBM) disease or Goodpasture syndrome is characterized by pulmonary hemorrhage, glomerulonephritis, or both. Pathognomonic to Goodpasture syndrome are autoantibodies to the renal glomerular and lung alveolar basement membrane. The specific autoantigen is the 235 amino acid carboxy-terminal noncollagenous domain of type IV collagen. Urinalysis of affected subjects reveals proteinuria, dysmorphic RBCs, WBCs, and RBC cellular and granular casts. Diagnostic testing includes the detection of anti–GBM antibodies. In cases in which only the presence of diffuse alveolar hemorrhage occurs, antibodies might not be present. In those cases the diagnosis is established by demonstrating linear immunofluorescence in lung tissue.
Patients presenting with renal involvement often have abrupt onset of oliguria or anuria, hematuria, and anemia. Renal biopsy can reveal crescents in more than 50% of the glomeruli on light microscopy (LM). Immunofluorescence demonstrates linear deposition of IgG along the glomerular capillaries and occasionally the distal tubules. Rarely, IgA or IgM can be present. Anti-GBM antibodies are also detected in the serum by means of immunofluorescence or ELISA. Many patients are also found to have positive results for ANCAs, particularly pANCA/MPO.
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Created: Dec 13, 2010.