A progressive, rare, and usually fatal autoimmune disorder affecting primarily the kidney and lung. It is characterized by glomerulonephritis and intra-alveolar haemorrhage. It begins as haemoptysis, anaemia, and dyspnoea, which are followed by hematuria and proteinuria. Haemoptysis is usually accompanied by the ”butterfly-wing” type of pulmonary infiltrates that radiate from each hilar region and are visible on x-rays. Weakness, cough, pallor, low-grade fever, and tachyardia are the additional symptoms. Pathologically, there are changes typical of glomerulonephritis, haemorrhages into the alveolar spaces, deposits of hemosiderin, and thickening of the alveolar septa.
It can present at any age and is most common in young men, with onset between 16 and 61 years of age (median 21 years). The glomerulonephritis is produced by antibodies against the glomerular membrane.
Death is usually due to renal failure. Aetiology unknown. Familial occurrence reported. The increased incidence in HLA-DRw2-carriers (relative risk 30 times higher) indicates the possibility of genetic factors.
- E. W. Goodpasture:
The significance of certain pulmonary lesions in relation to the etiology of influenza. American Journal of the Medical Sciences, Thorofare, N.J., 1919; 158: 863-870.