Antiglomerular basement membrane (Goodpasture) disease

Goodpasture disease is defined by the presence of autoantibodies to the alpha-3 chain of type IV collagen. It presents with rapidly progressive renal dysfunction often combined with pulmonary hemorrhage.

Simultaneous hemoptysis and renal failure (the pulmonary-renal syndrome) has many causes, but where the renal disease is a rapidly progressive glomerulonephritis, about 15% of cases will be Goodpasture disease, with the remainder mostly antineutrophil cytoplasmic antibody vasculitis.

Rapid diagnosis by early renal biopsy and serologic testing is essential, because appropriate treatment given before renal damage is advanced can result in an excellent prognosis.

Treatment is by removal of circulating antibodies using plasma exchange combined with agents to prevent their continued production.

Goodpasture disease, also known as antiglomerular basement membrane antibody (anti-GBM) disease, is an important cause of pulmonary-renal syndrome (Goodpasture syndrome).[1]Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003 Jul 7;64(7):1535-50. http://www.ncbi.nlm.nih.gov/pubmed/12969182?tool=bestpractice.com Pulmonary-renal syndrome consists of glomerulonephritis and pulmonary hemorrhage. Goodpasture disease is caused by an autoantibody to the alpha-3 chain of type IV collagen. This type of collagen is principally found in the basement membranes of alveoli and glomeruli.