The prognostic significance in Goodpasture's disease of specificity, titre and affinity of anti-glomerular-basement-membrane antibodies

Nephron Clin Pract. 2003;94(3):c59-68. doi: 10.1159/000072022.

Abstract

Background: The nephrotoxic potential of anti-glomerular-basement-membrane (GBM) antibodies has been demonstrated in numerous animal experiments. However, it is not known to what extent the properties of circulating anti-GBM antibodies in human disease reflect the severity of the disease and predict the outcome.

Methods: Clinical data were collected for 79 Swedish patients for whom a positive result had previously been obtained with anti-GBM ELISA. In stored sera from the patients, we measured antibody concentration, specificity and affinity together with antineutrophil cytoplasmic antibodies and alpha(1)-antitrypsin phenotype.

Results: Six months after diagnosis, 27 (34%) were dead, 32 (41%) were on dialysis treatment and only 20 (25%) were alive with a functioning native kidney. The best predictor for renal survival was renal function at diagnosis. In patients who were not dialysis dependent at diagnosis however, renal survival was associated with a lower concentration of anti-GBM antibodies, a lower proportion of antibodies specific for the immunodominant epitope and the histological severity of the renal lesion. The only factor that correlated with patient survival was age.

Conclusions: Immunochemical properties of autoantibodies do not affect patient survival in anti-GBM disease but seem to be a factor in renal survival in patients detected before renal damage is too advanced.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Aged
  • Anti-Glomerular Basement Membrane Disease / diagnosis*
  • Anti-Glomerular Basement Membrane Disease / drug therapy
  • Anti-Glomerular Basement Membrane Disease / enzymology
  • Anti-Glomerular Basement Membrane Disease / mortality
  • Antibodies / blood*
  • Antibodies / metabolism
  • Antibody Affinity*
  • Antibody Specificity*
  • Autoantibodies / biosynthesis*
  • Autoantibodies / blood*
  • Autoantibodies / metabolism
  • Autoantigens / immunology
  • Binding Sites, Antibody
  • Child
  • Child, Preschool
  • Collagen Type IV / immunology
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Middle Aged
  • Prognosis
  • Recombinant Proteins / immunology
  • Sexual Dysfunction, Physiological
  • alpha 1-Antitrypsin Deficiency / immunology

Substances

  • Antibodies
  • Autoantibodies
  • Autoantigens
  • Collagen Type IV
  • Recombinant Proteins
  • antiglomerular basement membrane antibody
  • type IV collagen alpha3 chain