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. Copyright 2003 S. Karger AG, Basel
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. Copyright 2003 S. Karger AG, Basel
Authors: Vadim Pedchenko; Olga Bondar; Agnes B Fogo; Roberto Vanacore; Paul Voziyan; A Richard Kitching; Jörgen Wieslander; Clifford Kashtan; Dorin-Bogdan Borza; Eric G Neilson; Curtis B Wilson; Billy G Hudson Journal: N Engl J Med Date: 2010-07-22 Impact factor: 91.245
Authors: Carmela P Menzi; Barbara S Bucher; Mario G Bianchetti; Gianluigi Ardissino; Giacomo D Simonetti Journal: Pediatr Res Date: 2018-01-10 Impact factor: 3.756