It presents with haemoptysis (in 60%) and severe progressive glomerulonephritis. Smoking appears to increase risk of intrapulmonary haemorrhage.
Chest radiography shows blotchy shadowing due to intrapulmonary haemorrhage. These features usually precede the glomerulonephritis by several weeks. Anti-glomerular basement antibodies are found in the serum. The titre of antibody is not related to rapidity of clinical deterioration. The serum complement level is usually normal.
If untreated, fewer than 15% of patients survive with useful renal function.
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