Goodpasture's syndrome

Alternative names:

anti-glomerular basement membrane antibody disease; glomerulonephritis-pulmonary hemorrhage; lung purpura-glomerulonephritis; pulmonary - renal syndrome; rapidly progressive glomerulonephritis with pulmonary hemorrhage

Definition:

A form of rapidly progressive glomerulonephritis (inflammation of the kidney glomeruli) involving progressive decrease in kidney function, accompanied by a cough with bloody sputum.

Causes, incidence, and risk factors:

The disorder is characterized by deposits of antibodies in the basement membranes of both the kidney glomerulus and the lung alveoli, causing both glomerulonephritis and pulmonary (lung) bleeding. The exact cause is unknown. It is an autoimmune disorder (action of the immune system against normal body tissues). Sometimes the disorder is triggered by a viral infection or by inhalation of gasoline or hydrocarbon solvents.

The disorder is rare, affecting approximately 1 out of 100,000 people. It is most often seen in men ages 16 to 61, usually around 20 years old. Smoking increases the risk of developing the disorder. It may develop after a recent viral respiratory infection. There is an inherited predisposition for Goodpasture's syndrome.

The disorder usually manifests with excretion of protein and blood in the urine. It progresses rapidly to kidney failure. Antibody deposits in the lungs cause bleeding within the lung tissues, resulting in the bloody sputum, which may be one of the first symptoms of the disorder. Iron deficiency anemia may be present, as well as anemia associated with renal failure, and may be worse than expected considering the amount of kidney damage.