Goodpasture Syndrome - can it be cured?
Most doctors for all their medical practice do not meet with such a disease as Goodpasture's syndrome. This is a very rare disease, which is manifested in 0.5 cases per million population. They are sick in most cases of young people, in the majority of recorded cases, men aged 12-35 years. The presence of bad habits( smoking, alcohol, drugs) on the occurrence or development of the disease is not affected. While the proven fact of a possible catalyst for the development of the disease is the poisoning with volatile vapors of heavy hydrocarbons. In fact, it is a severe immune disease of the lungs and kidneys that occur almost simultaneously. It is not surprising that these organs are the main filters of the body from harmful substances, so the first blow take over.
The first clinical manifestation of Goodpasture's syndrome is a characteristic cough with shortness of breath or hemoptysis. Such symptoms are easily confused with pneumonia or acute bronchial injury. After carrying out the X-ray, neoplasms of infiltrates of various sizes in the lungs can be noticed, often they arise precisely in the basal zones. After a complete analysis of the sputum, you can find an increased content of iron, which comes from the destroyed molecules of hemoglobin. Sometimes the disease is accompanied by fever, fever, but in many patients such symptoms are simply not observed. Most patients in the early stages of the disease have signs of glomerulonephritis( increased blood pressure, a decrease in the amount of daily urine volume, edema, detection of proteins and erythrocytes in the urine).
The course of the disease rarely leads to a complete recovery of the patient, perhaps only a temporary improvement in the general condition or a decrease in the effect of the disease on the human body. Each patient has a different course of the disease. With a primary lesion of the lungs or kidneys, the patient may die after the first penetrating bleeding, which simply does not have time to stop. In such cases, only operative surgical treatment is possible. In patients who were able to survive the first and most dangerous bleeding, the course of the disease may be weakened, but it will not be completely neutralized by local bleeding. Moreover, the patient's condition will worsen. When the kidneys are affected often the disease proceeds relatively easily, but often rapidly develops into an oliguric renal failure, from which patients die within a couple of months, sometimes - a couple of weeks. With improper or poor treatment of patients with Goodpasture's syndrome, they can live up to six months, less often longer.
Causes of the disease
The causes of the Goodpasture syndrome are not established accurately. There are suggestions that this may be a genetic disease that occurs when a HLA-DRW2 marker is present. There is an opinion that this is the result of a transferred virus infection( hepatitis A, flu, viral sore throat, meningitis) or when working in harmful industries( chemical organic industry, household aromatic chemistry production).Sometimes, some medications( for example, D-penicillamine) can lead to the development of the disease.
The main process in the tissues of the lungs and kidneys is the formation of autoantibodies in the capillaries of the kidneys and lungs. Some scientists believe that the formation of such autoantibodies can lead to prolonged smoking or inhalation of various carcinogenic substances.
Treatment of Good Supply Syndrome
The main condition for effective treatment of the disease is regular plasmapheresis, which is performed daily or every other day for several weeks. It is needed to replace aHTH-GBM antibodies. The effectiveness of the process is enhanced by the administration of glucocorticoids( methylprednisolone in combination with prednisolone and cyclophosphamide).This is done to prevent the emergence of new antibodies. Such therapy is sometimes reduced in the case of cessation of improvement in pulmonary and renal functions. The mortality of such patients often occurs in the first few weeks of the disease, when the dialysis is not just done by a doctor or if more than 50% of neuroses on a biopsy have occurred. Often in the early stages of the disease can be detected by sputum analysis after expectoration. It should be understood that quite a few diseases have similar symptoms with Goodpasture's syndrome, especially often it is confused with the latent forms of pneumonia. Patients who are ANCA-positive are easier to treat. But the continuation of smoking or an infection of the respiratory tract can lead to a relapse of the disease. It is interesting that patients who underwent transplantation of damaged kidneys are not insured against recurrent disease, so a relapse of the disease is possible in the transplant.
In case of pulmonary hemorrhage, anticoagulants are not allowed, but steroids can be used only in the absence of severe bleeding or the prerequisites for their occurrence. Repeated course of plasmapheresis proved to be very effective( complete replacement of blood plasma in one cycle).Such patients had the possibility of complete recovery of lung function and a positive change in kidney function.
In fact, modern medicine has been able to alleviate the course of the disease or to stave off a lethal outcome, but no real changes for the better. But the disease can be overcome, as evidenced by spontaneous remissions. Therefore, the disease must begin to fight in the early stages of detection and diagnosis of the disease, do not delay with treatment. Only then will it be possible to stop the disease or ease its course.
Diagnosis of Goodpasture Syndrome
Symptoms of Goodpasture Syndrome are similar to other diseases. For example, pulmonary hemorrhage with renal failure may occur in embolism, thrombophlebitis of various types, deep vein thrombosis or arteries, with circulatory failure with stagnation in the kidneys or lungs.
If suspected Goodpasture syndrome, a complete urine and sputum test is performed for the presence of protein and iron. It is noted that due to the large amount of iron in the lung sputum, it is possible to develop iron-deficiency diseases, a sharp increase in ESR.In the urine, proteins, red blood cells are detected, the density of urine sharply decreases. Perhaps an increase in the blood in the urine, a decrease in the iron content in the blood.
X-ray of the lungs can detect the fact of the development of the disease by the number and size of infiltrates in the radical region of both lungs, which can spread to other parts of the lungs. Examination of respiratory functions can reveal the fact of the disease, but it is possible only after a complete examination of the respiratory system. For the final confirmation of the diagnosis, an open biopsy of the renal and pulmonary tissues is performed.
When diagnosing Goodpasture's syndrome, attention is drawn to the presence of simultaneous pathologies of the lungs and kidneys with pronounced renal failure, respiratory dysfunction with hemoptysis. To be afraid of strong pulmonary or renal bleeding.