Summary
Goodpasture's disease is a very rare disease characterized by the association of pulmonary hemorrhage, extracapillary glomerulonephritis and anti-glomerular basement membrane antibodies. The annual incidence in Europe has been estimated at about 0.5 to 1 cases per one million inhabitants. The incidence higher in spring and early summer. The disease occurs most often in Caucasian populations and is slightly more frequent in males than in females. Goodpasture's disease is due to autoantibodies against the NC1 domain of the alpha3 chain of type IV collagen. The restricted distribution of this molecule explains why the disease affects only specific organs, such as lung and kidney. Autoreactive T cells also appear to play a role in the pathogenesis. Treatment is based on the association of corticosteroids, cyclophosphamide and plasma exchange. With this treatment, patient survival is about 75% at one year. Depending on the severity of renal failure and pulmonary hemorrhage, patients may require dialysis and/or respiratory support. It is essential to carefully avoid fluid overload and to rapidly treat infections. Renal survival at one year is higher than 90% for patients who are treated early, but is lower than 10% when patients are dialysis-dependent at the start of treatment. *Author: Dr J. Rossert (September 2005)*.
