anti GBM disease

Last reviewed 01/2018

Goodpasture's syndrome is a rare vasculitis characterised by the triad of:

• glomerulonephritis
• pulmonary haemorrhage
• autoantibodies to the glomerular basement membrane (GBM) and alveolar basement membrane (1,2)
  • antibodies bind in a linear pattern to a highly specific epitope of the *3 chain of type IV collagen of alveolar and glomerular basement membranes
  • immunoglobulins are almost exclusively of the IgG subtype (with only rare reports of non-IgG class anti-GBM antibodies)
  • IgM and IgA also may be found together with IgG along the GBM in approximately 10% to 20% of cases
  • anti-GBM antibodies may be found in the circulation by indirect immunoflourescence or radioimmunoassay

Goodpasture's syndrome occurs most frequently in young men and may cause renal failure, haemoptysis with pulmonary infiltrates and dyspnoea.

Reference:

• (1) A.H. Cohen and R.J. Glassock, Anti-GBM Glomerulonephritis including Goodpasture’s disease. In: C.C. Tisher and B.M. Brenner, Editors, Renal Pathology with Clinical and Functional Correlations ((ed 2) ed.),, Lippincott, Philadelphia, PA (1994), pp. 524–553.
• (2) Espinosa-Melendez E et al. Goodpasture’s syndrome treated with plasmapheresis. Arch Intern Med 1980;140: 542–543.