Goodpasture syndrome (GPS; also known as Goodpasture’s disease, anti-glomerular basement antibody disease, or anti-GBM disease) is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs 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.
Signs and Symptoms
The anti-glomerular basement membrane (GBM) antibodies primarily attack the kidneys and lungs, although, generalised symptoms like malaise, weight loss, fatigue, fever, and chills are also common, as are joint aches and pains
Lung symptoms usually antedate kidney symptoms and usually include: coughing up blood, chest pain, cough, and shortness of breath. Kidney symptoms usually include blood in the urine, protein in the urine, unexplained swelling of limbs or face, high amounts of urea in the blood, and high blood pressure.
Causes
Its precise cause is unknown, but an insult to the blood vessels taking blood from and to the lungs is believed to be required to allow the anti-GBM antibodies to come into contact with the alveoli.
- Exposure to organic solvents (e.g. chloroform) or hydrocarbons
- Exposure to tobacco smoke
- Certain gene mutations (HLA-DR15)
- Infection, such as influenza A
- Cocaine inhalation
- Metal dust inhalation
- Bacteraemia
- Sepsis
- High-oxygen environments
- Treatment with anti-lymphocytic