Anti-glomerular basement membrane antibody-induced glomerulonephritis.

Anti–glomerular basement membrane antibody-induced glomerulonephritis. Sixty-three glomerulonephritic patients with evidence of anti–glomerular basement membrane (GBM) antibody by immunofluorescence, elution of specific antibody and/or detection of circulating antibody are reviewed. Thirty-two of the patients had concomitant pulmonary manifestations (hemorrhage and/or pulmonary failure) compatible with Goodpasture's syndrome, 21 had only glomerulonephritis, and ten without prior immunopathologic studies had antiGBM antibodies in renal transplants and no history of pulmonary symptoms. The majority of the patients were young males who succumbed to the disease process or required dialysis and/or transplantation. Five males and two females did not develop permanent renal insufficiency. Bilateral nephrectomy (19 patients) was felt to be helpful or life-saving in seven patients; however, six patients had continued pulmonary symptoms after nephrectomy, contributing to death in three cases. Five of 13 nonnephrectomized or unilaterally nephrectomized individuals died in pulmonary failure. Immunosuppressive therapy was not generally helpful. Circulating antiGBM antibodies persisted for an average of six to eight months after bilateral nephrectomy. Transplantation was usually successful when delayed until circulating antiGBM antibodies had declined; however, evidence of recurrent glomerulonephritis developed in 19, possibly 20, of 34 transplanted patients, resulting in graft failure in seven, possibly eight, instances. Glomerulonephrite induite par des anticorps anti membrane basale. Soixante trois malades atteints de glomerulonephrite ou l'anticorps anti membrane basale (GBM) a ete mis en evidence par immunofluorescence, elution de l'anticorps specifique et/ou detection de l'anticorps circulant sont etudies. Trente trois de ces malades avaient des manifestations pulmonoaires concomitantes (hemorragie et/ou insuffisance respiratoire) compatible avec un syndrome de Goodpasture, 21 avaient une glomerulonephrite isolee, dix enfin, qui n'avaient pas eu d'etude immunopathologique anterieure, avaient des anticorps anti GBM dans des reins transplantes et aucun symptome pulmonaire. La majorite des malades etaient de jeunes hommes qui decederent du fait de la maladie ou durent etre hemodialyses et/ou transplantes. Cinq hommes et deux femmes n'eurent pas d'insuffisance renale. La nephrectomie bilaterale (19 malades) fut jugee utile ou indispensable chez sept malades. Cependant six d'entre eux eurent des symptomes pulmonaires persistants apres la nephrectomie, manifestations qui contribuerent a la mort dans trois cas. Cinq parmi les 13 malades non nephrectomises ou unilateralement nephrectomises moururent d'insuffisance pulmonaire. Le traitement immunosuppresseur n'a pas ete favorable dans l'ensemble. Les anticorps anti GBM circulants ont persiste six a huit mois, en moyenne, apres la nephrectomie bilaterale. La transplantation a reussi habituellement quand elle a ete retardee jusqu'a la diminution des anticorps circulants anti GBM. Cependant des signes de glomerulonephrite recurrente sont apparus chez 19, et peut-etre 20, parmi les 34 malades transplants, conduisant a l'echec du transplant dans sept cas et peut-etre huit.