Goodpasture syndrome
| Goodpasture syndrome | |
|---|---|
| Other names | Goodpasture's syndrome, Goodpasture disease, Goodpasture's disease, anti–glomerular basement membrane disease, anti–glomerular basement membrane antibody disease, anti-GBM disease, anti-GBM antibody disease |
| Micrograph of a crescentic glomerulonephritis that was shown to be anti–glomerular basement membrane disease, PAS stain | |
| Specialty | Nephrology, pulmonology, immunology |
Goodpasture syndrome (GPS), also known as anti–glomerular basement membrane disease, is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs, glomerulonephritis, and kidney failure. It is thought to attack the alpha-3 subunit of type IV collagen, which has therefore been referred to as Goodpasture's antigen. Goodpasture syndrome may quickly result in permanent lung and kidney damage, often leading to death. It is treated with medications that suppress the immune system such as corticosteroids and cyclophosphamide, and with plasmapheresis, in which the antibodies are removed from the blood. Due to the GPS's rapid progression, the significant difficulty of treating the disease is identifying it early and making the appropriate response before severe damage occurs to the kidneys and or lungs. Consequently, the standard treatment plan of corticosteroids, cyclophosphamide, and plasmapheresis is vigorous and fast-acting, including high plasma volume exchange and an intensive dose of corticosteroid and cyclophosphamide based on the patient's body weight in kilograms.
The disease was first described by an American pathologist Ernest Goodpasture of Vanderbilt University in 1919 and was later named in his honor.