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Goodpasture Syndrome

作者信息

Rout Preeti, DeVrieze Bradley W.

机构信息

Wilson Case Western University

Creighton University School of Medicine

Abstract

Goodpasture syndrome refers to an anti-glomerular basement membrane (anti-GBM) disease that involves both the lungs and kidneys, often presenting as pulmonary hemorrhage and glomerulonephritis. Belonging to 1 of the 3 main causes of crescentic glomerulonephritis, Goodpasture syndrome often has the worst prognosis, especially when not promptly treated. Please see our companion review, "Rapidly Progressive Glomerulonephritis," for further information and classification of Goodpasture syndrome in the context of the other rapidly progressive glomerulonephritides. Some clinicians may interchangeably use several terms, including anti-glomerular basement membrane disease, Goodpasture syndrome, and Goodpasture disease.[1] Anti-GBM disease can also refer to the presence of renal disease without pulmonary involvement, while Goodpasture disease or syndrome always has pulmonary involvement. Some clinicians even describe coexistent renal and pulmonary disease of any kind as "Goodpasture Syndrome," but in general, this term refers to anti-GBM antibodies. About 40% to 60% of cases are associated with alveolar hemorrhage, while fewer than 10% of anti-GBM disease cases present with isolated pulmonary involvement.[3] Goodpasture syndrome is an eponym named after Ernest Goodpasture, who first described this disorder in 1919. Immunofluorescence techniques developed in the 1960's and the discovery of anti-GBM antibodies further led to a better understanding of the pathogenesis of Goodpasture syndrome.[4]

摘要

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