- Goodpasture's syndrome (pulmonary-renal syndrome) - causes and risk factors
- Goodpasture's syndrome (pulmonary-renal syndrome) - symptoms
- Goodpasture's syndrome (pulmonary-renal syndrome) - diagnosis
- Goodpasture's syndrome (pulmonary-renal syndrome) - treatment
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Goodpasture's syndrome, or pulmonary-renal syndrome, is a rare autoimmune disease that affects the kidneys and lungs. Goodpasture's syndrome can be life threatening as it can lead to respiratory and / or kidney failure very quickly, even within a few days. What are the causes and symptoms of Goodpasture's syndrome? What is the treatment?
Goodpasture's syndrome , otherwisepulmonary-renal syndrome,belongs to the group of diseases known as systemic vasculitis. In the course of Goodpasture's syndrome, the basement membrane of the glomerular vessels and alveoli become inflamed, resulting in bleeding into the alveoli and rapidly progressive glomerulonephritis. The disease is life-threatening because very quickly, even within a few days, it can lead to respiratory failure and / or renal failure.
Goodpasture's syndrome is rare (incidence in Europe - about 1/1 million inhabitants / year), most often in people between 20 and 30 years of age and in those aged 50-65. The disease is more often diagnosed in men than in women.
Goodpasture's syndrome (pulmonary-renal syndrome) - causes and risk factors
Goodpasture's syndrome is an autoimmune disease. In this type of disease, the immune system recognizes the body's own cells as foreign and produces antibodies against them that attack them and cause them to become inflamed and destroyed. In the course of Goodpasture's syndrome, antibodies (anti-GBM) are produced against the basement membrane of the glomerular vessels and alveoli. Antibody damage to the blood vessels of the glomeruli results in glomerulonephritis, which can lead to chronic renal failure. In turn, damage to the blood vessels of the alveoli leads to bleeding.
Risk factors are: exposure to hydrocarbons and tobacco smoke, inhalation of cocaine and infections.
Goodpasture's syndrome (pulmonary-renal syndrome) - symptoms
Symptoms of lung involvement by disease:
- shortness of breath - initially it may be exercise-induced, then paroxysmal at rest
- dry cough
- hemoptysis
- sometimescyanosis
Symptoms of kidney involvement by the disease:
- hematuria
- problems with urination (e.g. oliguria)
- swelling all over the body
- hypertension
- pain in the lumbar region
In addition, general symptoms, usually flu-like, may appear, e.g. fatigue, fever, pain in muscles and joints, lack of appetite.
Goodpasture's syndrome (pulmonary-renal syndrome) - diagnosis
Blood tests are performed to detect anti-GBM antibodies. In the case of Goodpasture's syndrome, it is also found, inter alia, elevated levels of creatinine and urea in the blood, indicating kidney damage.
It is also necessary to test the urine, which shows, inter alia, presence of protein or red blood cells.
The doctor also tests the respiratory system. During the stethoscope examination, you can hear crackles, and sometimes even a bronchial murmur. On the other hand, the chest X-ray shows speckled shadows that suggest inflammatory changes.
In some cases, a kidney or lung biopsy may be necessary.
Goodpasture's syndrome (pulmonary-renal syndrome) - treatment
Patients are given glucocorticosteroids and cyclophosphamide. In addition, plasmapheresis is performed - a procedure that cleans the blood of unwanted elements - in this case, anti-GBM antibodies.
In some patients, the kidney starts working properly, usually in the first weeks of treatment. However, 40-50 percent. despite treatment, the kidney does not regain its functions. Then, renal replacement therapy is needed in the form of hemodialysis. After a few months, the patient's condition may improve and hemodliosis may not be needed. However, doctors' experience has shown that recovery of kidney function at a later date is rare. If the kidney does not start working properly during the first weeks of treatment and the patient needs renal replacement therapy, this usually means that the renal failure is irreversible.
However, you can prevent the progression of kidney failure and even its development. It is enough to diagnose the disease as early as possible and apply appropriate treatment.