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Goodpasture’s syndrome (also known as Goodpasture’s disease and anti-glomerular basement membrane disease) is a rare condition characterised by rapid destruction of the kidneys and haemorrhaging of the lungs. Although many diseases can present with these symptoms, the name Goodpasture’s syndrome is usually reserved for the autoimmune disease produced when the patient’s immune system attacks cells presenting the Goodpasture antigen (a type II hypersensitivity reaction), which are found in the kidney and lung, causing damage to these organs. The disease bears the name of the American pathologist Dr. Ernest Goodpasture, whose 1919 description is regarded as the first report on the existence of the condition.[1][2]
Goodpasture’s syndrome can cause people to cough up blood or feel a burning sensation when urinating. But its first signs may be vague, such as fatigue, nausea, difficulty breathing, or paleness. These signs are followed by kidney involvement, represented first by small amounts of blood in the urine, protein in the urine, and other clinical and laboratory findings.[3]
Other patients present with both lung and kidney disease; however, some patients present with one of these diseases alone. The first lung symptoms usually develop days to months before kidney damage is evident.
Possible causes, among them the presence of an inherited component; exposure to certain chemicals, including hydrocarbon solvents and the weed killer Paraquat; and viral infections.[3]
Lung damage may cause nothing more serious than a dry cough and minor breathlessness and such mild symptoms may last for many years before more severe ones develop. At its most serious, however, lung damage may cause severe impairment of oxygenation so that intensive care is required. Deterioration between the two extremes may occur very rapidly, often at the same time as rapid deterioration in the kidney. The patient often does not seek medical attention until he or she begins coughing up blood (hemoptysis). The patient may be anemic due to loss of blood through lung haemorrhaging over a long period. In Goodpasture’s syndrome, unlike many other conditions that cause similar symptoms, lung hemorrhaging most often occurs in smokers and those with damage from lung infection or exposure to fumes.
The kidney disease mostly affects the glomeruli causing a form of nephritis. It is usually not detected until a rapid advance of the disease occurs so that kidney function can be completely lost in a matter of days, a condition known as rapidly progressive (Crescentic) glomerulonephritis, or RPGN. Blood leaks into the urine causing hematuria, the volume urinated decreases and urea and other products usually excreted by the kidney are retained and build up in the blood. This is acute renal failure. Renal failure does not cause symptoms until more than 80% of kidney function has been lost. Symptoms include loss of appetite and sickness at first and then, when the damage is more advanced, breathlessness, high blood pressure and edema (swelling caused by fluid retention). The kidney involvement usually presents as nephritic syndrome, i.e. hematuria, a reduced glomerular filtration rate, and high blood pressure. This is in contrast to nephrotic syndrome, a more rare outcome of Goodpasture’s, characterized by an abnormally large amount protein in the urine (proteinuria), coupled with severe edema.
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