Goodpasture's syndrome

Benoit, F.L.; Rulon, David B.; Theil, George B.; Doolan, Paul D.; Watten, Raymond H.

doi:10.1016/0002-9343(64)90199-8

Cited by 188 publications

(26 citation statements)

References 23 publications

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“…Actually there were only 2 young men with pulmonary haemorrhage in the entire cohort. The young age and large male preponderance in early series might have been influenced by the fact that some cohorts emanated from US military facilities [3, 4]. …”

Section: Discussionmentioning

confidence: 99%

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The Prognostic Significance in Goodpasture’s Disease of Specificity, Titre and Affinity of Anti-Glomerular-Basement-Membrane Antibodies

2004

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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 α₁-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.

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Section: Discussionmentioning

confidence: 99%

“…Over the following decade, the notion of Goodpasture’s syndrome as a distinct disease entity developed, and the pathological process was supposed to be driven by an autoimmune mechanism [3, 4]. The concept of nephrotoxic serum causing glomerulonephritis had already been developed at the turn of the century by Lindeman [5].…”

Section: Introductionmentioning

confidence: 99%

The Prognostic Significance in Goodpasture’s Disease of Specificity, Titre and Affinity of Anti-Glomerular-Basement-Membrane Antibodies

2004

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“…NTGN induced by anti-GBM Ab represents an experimental model for human Goodpasture's syndrome (14). Patients suffering from this type of GN show rapidly progressive renal lesions and severe hemorrhagic pulmonary involvement.…”

Section: Discussionmentioning

confidence: 99%

“…One is the deposition of Ag-Ab ICs preformed in circulation or on the wall of glomerular capillaries, and the other is the emergence of autoAbs against GBM (10) or other renal constituents such as renal tubular Ags. The latter GN can be induced by injecting anti-GBM Ab into rats, rabbits, and mice, and this is known as Masugi nephritis or nephrotoxic serum glomerulonephritis (NTGN) and serves as a model for human Goodpasture ne-phritis (11)(12)(13)(14)(15)(16). On the other hand, there has been accumulating data that the development of GN is independent of Ab and dependent on T cell-mediated responses, particularly the delayed-type hypersensitivity (DTH) mechanism (17)(18)(19)(20).…”

Section: Introductionmentioning

confidence: 99%

Resistance of Fc receptor- deficient mice to fatal glomerulonephritis.

Park¹,

Ueda²,

Ohno³

et al. 1998

J. Clin. Invest.

249

191

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Immune complex-mediated inflammation is a common mechanism of various autoimmune diseases. Glomerulonephritis (GN) is one of these diseases, and the main mechanism of the induction of GN has been unclear. We examined the contribution of Fc receptors in the induction of nephrotoxic GN by establishing and analyzing mice deficient in the Fc receptor ␥ chain (FcR ␥ ). Whereas all wild-type mice died from severe glomerulonephritis with hypernitremia by administration of anti-glomerular basement membrane (GBM) antibodies, all FcR ␥ -deficient mice survived. Histologically, wild-type mice showed glomerular hypercellularity and thrombotic changes, whereas the renal tissue in FcR ␥ -deficient mice was almost intact. Deposition of anti-GBM antibody as well as complement components in the GBM were equally observed in both wild-type and knockout mice. These results demonstrate that the triggering of this type of glomerulonephritis is completely dependent on

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“…Subsequently, the clinical syndrome of rapidly progressive renal failure with crescentic glom erulonephritis, pulmonary hemorrhage, iron deficiency anemia, and no systemic disease occurring largely in young men was further characterized by the presence of antibody to glomerular and alveolar basement membrane [11 ]. This complete set of conditions is now rather uniformly re ferred to as Goodpasture Until the advent of chronic dialysis, Goodpasture's syn drome was regarded as an invariably fatal illness with one half of the deaths from pulmonary insufficiency and the remainder from uremia [2]. Dialytic therapy prevented death from renal failure and made possible the observation both immunologic and degenerative diseases of basement that bilateral nephrectomy was frequently an effective means of terminating pulmonary hemorrhage.…”

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confidence: 99%

Glomerular Basement Membrane and Anti-GBM Antibody Disease

Kirschbaum¹

1981

Nephron

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The GBM is an important structure for the development of immunologic renal disease. It may serve as the primary antigen or as the site of deposition of immune complexes. The GBM is recognized as the major protein filtration barrier of the glomerulus. The composition of the GBM includes collagen, predominantly but not exclusively type IV, which is present as procollagen. Two glycoproteins, laminin and fibronectin, are either intrinsic components of the GBM or intimately associated with the membrane. Heparan sulfate, a glycosaminoglycan, is contained within the substance of the GBM and seems to comprise the primary anion of the structure. Antibodies to these individual GBM components are being recognized with increasing frequency in patients with glomerulonephritis. The recognition of these antibodies may serve to expand our definition of both immunologic and degenerative diseases of basement membrane.

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Goodpasture's syndrome

Cited by 188 publications

References 23 publications

The Prognostic Significance in Goodpasture’s Disease of Specificity, Titre and Affinity of Anti-Glomerular-Basement-Membrane Antibodies

The Prognostic Significance in Goodpasture’s Disease of Specificity, Titre and Affinity of Anti-Glomerular-Basement-Membrane Antibodies

Resistance of Fc receptor- deficient mice to fatal glomerulonephritis.

Glomerular Basement Membrane and Anti-GBM Antibody Disease

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