Antibody-negative Goodpasture’s disease

Goodpasture’s syndrome is a rare, life-threatening pulmonary-renal syndrome characterized by the production of autoantibodies to a component of the glomerular basement membrane (GBM). The syndrome involves an autoimmune response to the non-collagen domain 1 of the human alpha-3 chain of type IV collagen, α3(IV)NC1. The term Goodpasture’s syndrome is often used to refer to the constellation of a rapidly progressive (crescentic) glomerulonephritis, pulmonary haemorrhage and, by definition, detection of circulating anti-GBM antibody. Many clinicians rely on commercially available assays to detect anti-GBM antibodies in the clinical setting of rapidly progressive glomerulonephritis; however, there are few case reports of Goodpasture’s syndrome in the absence of these antibodies. We report here an unusual case of a patient with clinical and histological evidence of antiGBM antibody mediated Goodpasture’s syndrome with positive anti-neutrophil cytoplasm antibodies (ANCA) but without evidence of circulating anti-GBM antibody. Case report