Goodpasture’s syndrome is a rare autoimmune disease that damages the lining of the kidneys and lungs. The condition can lead to life threatening symptoms, including organ failure.
Goodpasture’s syndrome is a rare disease that occurs when the immune system mistakenly produces antibodies that damage the lining of the kidneys and lungs. In some cases, only the kidneys become involved. Without quick treatment, Goodpasture’s syndrome can lead to kidney and lung failure and become life threatening.
The name Goodpasture’s syndrome derives from the doctor who first discovered the disease. However, doctors commonly refer to Goodpasture’s syndrome as anti-glomerular basement membrane (anti-GBM) disease.
Anti-GBM disease is the
It occurs when the immune system attacks the glomerular and alveolar basement membranes. The basement membrane is a cellular structure that provides tissue support and filtration for the organs.
Anti-GBM disease involves the immune system producing antibodies that damage the basement membranes. In the kidneys, anti-GBM antibodies affect the tiny structure that helps filter water and waste. In the lungs, the antibodies affect the small air sacs called alveoli.
Anti-GBM disease is very rare, with about
For unknown reasons, the condition tends to most often develop in two different age groups. These include
Researchers do not know the exact cause of Goodpasture’s syndrome. It occurs when the immune system produces antibodies that target a type of collagen present in the kidneys and lungs. This immune reaction leads to inflammation of the kidneys and lungs.
Doctors think a combination of genes and the environment may trigger Goodpasture’s syndrome. Certain environmental factors may
- exposure to inhaled hydrocarbons, which are chemicals that derive from petroleum in products such as paint thinner, gasoline, and furnish polish
- inhaled cocaine
- contact with metal dust
- alemtuzumab, which is a medication for treating multiple sclerosis
Goodpasture’s syndrome can start with general symptoms and progress to symptoms relating to kidney and lung inflammation. Possible symptoms include:
- pale skin
- lack of appetite
Lung and kidney-related symptoms occur as inflammation and damage to the organs develop. Symptoms can include:
- high blood pressure
- trouble urinating
- swelling in the feet and legs
- pink colored urine due to blood
- foamy urine
- dry cough
- chest pain
- shortness of breathing
- coughing up blood
Complications can occur quickly due to Goodpasture’s syndrome without treatment. As the disease progresses, about
Kidney inflammation can lead to complications, including kidney failure, which may require short- or long-term dialysis. Typically, under
Doctors diagnose Goodpasture’s syndrome according to a person’s symptoms, medical history, physical exam, and diagnostic tests.
A kidney biopsy, when doctors remove a tissue sample, is the
Additional diagnostic tests also help rule out other conditions and indicate a need for a kidney biopsy. Tests may include:
- a urinalysis to check for blood in the urine
- kidney function tests to determine damage
- blood tests to reveal anemia due to blood loss
- a chest X-ray to identify bleeding in the lungs
The tests above are less invasive than a kidney biopsy and typically help provide clues to the diagnosis.
Treatment for Goodpasture’s syndrome aims to decrease inflammation, stop the production of the antibodies causing damage, and remove the existing anti-GBM antibodies.
Treatment may include medications, such as corticosteroids and immunosuppressants. Corticosteroids reduce inflammation and aid in controlling lung bleeding, while immunosuppressants help suppress atypical reactions from the immune system.
Doctors may also recommend plasma exchange, which allows the removal of the antibodies attacking the membranes.
The process involves using a special machine to separate the plasma from the blood. Once the separation occurs, the machine removes antibodies causing the damage. After treating the plasma, the machine returns it to the individual.
Goodpasture’s syndrome, also known as anti-glomerular basement membrane disease, is a rare autoimmune condition. It develops when the immune system atypically makes antibodies that attack the membranes in the kidney and the lungs. The cause remains unknown, but genes and the environment likely play a role in developing the disease.
The condition leads to inflammation in the lungs and kidneys, which can cause complications, including kidney and respiratory failure. Treatment includes medication and plasma exchange to stop inflammation and remove the antibodies from the blood.