title: The prognostic significance in Goodpasture's disease of specificity, titre and affinity of anti-glomerular-basement-membrane antibodies.
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.
authors.affiliation: Department of Nephrology, Lund University, Lund, Sweden. marten.segelmark@njur.lu.se
authors.forename: Mårten
authors.initials: M
authors.lastname: Segelmark
authors.forename: Thomas
authors.initials: T
authors.lastname: Hellmark
authors.forename: Jörgen
authors.initials: J
authors.lastname: Wieslander
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000201:enzymology / Q000401:mortality; D000906:Antibodies / Q000097:blood* / Q000378:metabolism; D000915:Antibody Affinity; D000918:Antibody Specificity; D001323:Autoantibodies / Q000096:biosynthesis* / Q000097:blood* / Q000378:metabolism; D001324:Autoantigens / Q000276:immunology; D001666:Binding Sites, Antibody; D002648:Child; D002675:Child, Preschool; D024141:Collagen Type IV / Q000276:immunology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011994:Recombinant Proteins / Q000276:immunology; D012735:Sexual Dysfunction, Physiological; D019896:alpha 1-Antitrypsin Deficiency / Q000276:immunology
chemical_list: D000906:Antibodies; D001323:Autoantibodies; D001324:Autoantigens; D024141:Collagen Type IV; D011994:Recombinant Proteins; C051951:antiglomerular basement membrane antibody; C057112:type IV collagen alpha3 chain