Glomerular basement membrane autoantibodies

An anti-glomerular basement membrane (GBM) disease is defined by an acute and rapid progressive glomerulonephritis in the presence of anti-GBM antibodies (Ab). In up to 70% of patients, pulmonary hemorrhages are present and the triple association (glomerulonephritis, pulmonary hemorrhage, and anti-GBM Ab) is referred to as Goodpasture syndrome. The main targets of pathogenic anti-GBM Ab are cryptic and present within the NC1 domain of the α3-chain of collagen IV. IgG1 and IgG3 anti-GBM Ab are predominant, and anti-GBM Ab are detected in the serum and/or in the histologic specimen of the kidney or the lung. Glomerulonephritis is linked with the level of circulating anti-GBM Ab and is fatal if untreated. Anti-GBM Ab determination is useful to monitor the treatment, and anti-GMB Ab disappearance is indicative of therapeutic success.