Goodpaste syndrome

The combination of hemosiderosis of the lungs and glomerulonephritis is Goodpasture's syndrome. Family cases of the disease are known. Cases of the disease in identical twins are described.

Goodpasture syndrome is characterized by immunological damage to the lungs and kidneys. This syndrome is referred to as diseases of immune complexes. It is believed that under the influence of infectious or toxic effects, pulmonary tissue acquires the signs of an antigen. The resulting antibodies are deposited in the kidneys and lung tissue, thereby damaging these organs.

Disease is more common in males. The main clinical manifestations of the disease are lung damage( dyspnea, cough, hemoptysis) and the appearance of blood in the urine( hematuria).In the initial stages, there is usually a microhematuria detected by the eye of hematuria is extremely rare.

General activities. Often urgent assistance is required - transfusion of blood components, removal of water-electrolyte disorders, inhalation of oxygen, intubation of the trachea, ventilation, peritoneal dialysis and hemodialysis.

Immunosuppressive therapy: conduct plasmapheresis, simultaneously with it prescribe pentoniosone cyclophosphamide. Plasmapheresis removes antibodies from serum, and corticosteroids and immunosuppressants suppress the production of these antibodies. A few days or weeks after the start of treatment, the radiographic picture and the parameters of the external respiration function are normalized. CRF can progress even on the background of treatment. Monotherapy with corticosteroids is effective in pulmonary hemorrhage, but does not affect the course of glomerulonephritis.

In the treatment of pulmonary hemosiderosis and Goodpasture syndrome, hormonal and immunosuppressive drugs are used. In severe renal manifestations, the methods of choice are hemodialysis or removal of the kidney followed by a donor organ transplant. In connection with anemia, iron-containing preparations, blood transfusion are recommended.

Forecast. In the absence of treatment, 75% of patients die from chronic renal failure, 25% from pulmonary hemorrhage. The same goes for the wrong treatment: the use of immunosuppressants in low doses or a short course. However, early plasmapheresis and long-term treatment with prednisone and cyclophosphamide significantly improve the condition of patients and somewhat reduce the lethality.