Study Examines Activity in Goodpasture's Disease

Analysis included patients with Goodpasture's disease, Alport's post-transplantation nephritis

WEDNESDAY, July 21 (HealthDay News) -- New findings suggest that Goodpasture's disease -- which is marked by progressive glomerulonephritis and pulmonary hemorrhage -- may involve a so-called autoimmune "conformeropathy," according to research published in the July 22 issue of the New England Journal of Medicine.

Vadim Pedchenko, Ph.D., of the Vanderbilt University Medical Center in Nashville, Tenn., and colleagues analyzed circulating antibodies in 57 patients with Goodpasture's disease, as well as kidney-bound antibodies in 14 patients with Goodpasture's disease and two patients with Alport's post-transplantation nephritis.

The researchers found that, in Goodpasture's disease, autoantibodies to the α3NC1 monomer and antibodies to the α5NC1 monomer were bound in the kidneys and lungs, suggesting that these monomers could serve as autoantigens. Elevated antibody titers upon diagnosis of anti-glomerular basement membrane disease were linked to later renal function loss. Though antibodies bound to epitopes that encompassed region EA in the α5NC1 monomer and EA and EB in the α3NC1 monomer, they didn't bind to native cross-linked α345NC1 hexamer, the authors write. In patients with Alport's, alloantibodies did bind to the EA region of the α5NC1 subunit in the intact hexamer, and the binding was reduced upon dissociation.

"In summary, this article clarifies the probable pathogenic role of anti-α5NC1 autoantibodies in Goodpasture's disease and Alport's post-transplantation nephritis, establishes more clearly the difference between the epitopes identified by Goodpasture's disease autoantibodies and Alport's post-transplantation nephritis alloantibodies, and further advances our understanding of the conformational nature of the Goodpasture's disease epitopes," writes the author of an accompanying editorial.

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