Goodpasture Syndrome - Causes, Symptoms and Treatment. MF.
Goodpasture syndrome is a disease that occurs when there is a defect in the immune system and is characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis( inflammation of the kidney tissue).
The incidence of the disease is 1 case per 2 000 000 population. The first peak incidence falls on the age of 20-30 years, with mainly men being ill, the second peak age is 50-60 years, both sexes are ill with the same frequency.
Causes of Goodpasture Syndrome
The cause of Goodpasture's syndrome is unknown. There are indications that the disease is associated with a viral infection, especially the influenza virus. Some environmental factors, such as organic solvents, gasoline, a number of drugs, can become predisposing factors in the development of the disease. It is known that pulmonary hemorrhage occurred, as a rule, in smokers, that is, the previous lung injury also plays a big role in the onset of the disease.
Goodpasture syndrome is based on the formation of specific proteins - antibodies specific for vascular cells. Under the action of antibodies occurs activation of complex chemical processes, which ultimately lead to damage to the vascular wall.
Symptoms of Goodpasture Syndrome
For the onset of the disease is characterized by the development of nonspecific symptoms in the form of weakness, drowsiness, increase in body temperature to 37-37.5 degrees, weight loss. Sometimes the first sign of the disease is a low concentration of hemoglobin in the absence of any bleeding.
Goodpasture syndrome is characterized by lung and kidney damage.
• Lung disease, as a rule, is manifested for the first time by hemoptysis( appearance of blood veins in the sputum, as well as individual spasms of liquid or partially curdled blood during a thawing).
This symptom is usually several months ahead of the kidney damage. Hemoptysis may be permanent or occur intermittently. In addition, the patient is concerned about pain in the chest, dyspnea at rest or with exercise, coughing.
Especially dangerous is the development of pulmonary hemorrhage. This condition is characterized by one-stage coughing of more than 100 ml of liquid blood. If large vessels are affected, bleeding can become potentially life-threatening. Even a slight hemoptysis, not to mention bleeding, leads to a decrease in hemoglobin and the development of iron deficiency anemia. Very often lung damage is complicated by pneumonia( pneumonia) or pulmonary edema( fluid accumulation in the lung tissue above the physiological norm, characterized by manifestations of respiratory failure).
• In case of kidney damage glomerulonephritis develops. In the onset of the disease in the urine appears a moderate amount of protein and erythrocytes. Massive proteinuria or edematous syndrome does not have time to develop, since the peculiarity of the disease is its rapidly progressive nature. Within a few weeks, the amount of urine sharply decreases and terminal renal failure is formed with pronounced impairment of kidney function.
Diagnosis of Goodpasture Syndrome
Diagnosis of Goodpasture Syndrome is based on the determination of antibodies to vascular cells by enzyme immunoassay. In the general analysis of blood, attention is drawn to the decrease in hemoglobin concentration as a consequence of blood loss, increase in the number of leukocytes and acceleration of the rate of erythrocyte sedimentation, which reflects the presence of inflammation. With lesions of the lungs on the roentgenogram, foci of infiltration are determined. These are areas of lung tissue with hemorrhages.
Treatment of Goodpasture Syndrome
Treatment of Goodpasture Syndrome should be timely and intensive. Hormonal preparations( prednisolone) and cytostatics( cyclophosphamide) are used. Transfusion of the erythrocyte mass and plasma is performed with the substitution purpose. With the development of terminal renal failure, hemodialysis sessions are performed.
Unfortunately, in about 40% of cases, treatment is ineffective, and patients die from pulmonary hemorrhage or from poisoning with toxic substances in violation of the excretory function of the kidneys. Currently, kidney transplantation( transplantation) does not contribute to reducing mortality.
Doctor therapist, nephrologist Sirotkina EV