anti-GBM Nephritis Research Articles

Variant "Goodpasture's syndrome"? The need for immunologic criteria in rapidly progressive glomerulonephritis and hemorrhagic pneumonitis.

The immunopathogenic mechanisms in three men with rapidly progressive glomerulonephritis and hemorrhagic pneumonitis were studied. Nephritis and fatal pulmonary hemorrhage occurred simultaneously in two patients. Typical Goodpasture's syndrome was defined in one by the presence of noncomplement-fixing IgG antiglomerular basement membrane (GBM) antibodies in his circulation, kidney, and lung. Immunofluorescent studies were negative in the second patient, although an immune complex pathogenesis was suggested by the presence of mixed cryoglobulinemia, decreased serum complement, and arteritis. The third patient also had anti-GBM-mediated nephritis, but his ultimately fatal pulmonary disease did not develop until after nephrectomy. Recurrence of the same anti-GBM nephritis was documented in his renal transplant at necropsy. It is suggested that "Goodpasture's syndrome" be restricted to patients with anti-GBM antibody-mediated nephritis and concomitant hemorrhagic pneumonitis.

The Presence of Anti-Glomerular Basement Membrane Antibodies in Peripheral Blood

Abstract The use of immunofluorescent assay for circulating anti-glomerular basement membrane (anti GBM) antibodies is described. Groups of people studied for the presence of anti-GBM antibody included: a) normals; b) immune-complex glomerulonephritic patients; c) anti-GBM antibody-mediated nephritic patients; d) a hemodialysis-maintained group of kidney disease patients; and e) an allergy clinic group. Anti-GBM antibodies were found in the circulation only in patients with anti-GBM antibody-mediated nephritis. Paired-label isotopic studies to determine the percentage fixation of kidney-fixing antibodies, combined with serial dilution of the same anti-GBM antibody-containing sera, allowed estimation of the sensitivity of the assay to be in the range of 0.1 to 1.0 µg/ml antibody content. Despite the sensitivity of the technique and its specificity, two principal handicaps were encountered: a) only 40% of people known to have anti-GBM antibody-mediated glomerulonephritis were shown to have circulating anti-GBM antibodies, and b) several sera were encountered which fixed with wide anatomical definition to the target substrate and made it difficult to recognize the presence of anti-GBM antibodies in such sera.