Goodpastures Syndrome

Juan Saus, Instituto de Investigaciones Citológicas de la Fundación Valenciana de Investigaciones Biomédicas and Departamento de Bioquímica y Biología Molecular de la Universität de Valencia, Valencia, Spain

Copyright © 1998 Elsevier Ltd. All Rights Reserved.

Since Ernest Goodpasture described the coexistence of hemoptysis and progressive renal failure in a young man after an episode of influenza in 1919, important advances have been made in our understanding of this unusual and, so far, exclusively human disease. Milestones have been the finding that the major pathogenic event is the linear deposition of circulating immunoglobulin G (IgG), the GP (Goodpasture) antibodies, along glomerular and alveolar basement membranes and the identification of the GP antigen as the C-terminal region of the a3 chain of basement membrane collagen IV.

Today the definitive diagnosis of GP syndrome is based on the detection of circulating antibodies against a3(IV)NCl in patients with renal-pulmonary involvement. Evidence suggests that the unique manner in which these high-affinity antibodies bind to the glomerular basement membrane efficiently interferes with plasma filtration. Restricted regions at both ends of the antigen are involved in antibody binding and in the formation of the GP epitope. Exclusively in humans, the N-terminal portion of this epitope can be phosphorylated and exists in multiple alternative antigen-related products that vary in their ability to be phosphorylated. In predisposed individuals, disturbance of these or other human-restricted processes may cause changes in the GP antigen peptide presentation and thereby initiate the autoimmune response.

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