Goodpasture's syndrome

August 12, 2017 17:51 | Genetic Diseases

combination hemosiderosis of the lungs and glomerulonephritis is Goodpasture's syndrome.Known family history of the disease.Cases of the disease in monozygotic twins.

Goodpasture's syndrome is characterized by immunological damage to the lungs and kidneys.This syndrome refers to diseases of immune complexes.It is believed that under the influence of infectious or toxic effects lung tissue gets antigen features.The resulting antibodies are deposited in the kidneys and lung tissue, thereby damaging these organs.

The disease is more common in males.The main clinical manifestations of the disease are pulmonary disease (dyspnea, cough, coughing up blood), and blood in the urine (hematuria).In the early stages usually takes place microscopic hematuria, hematuria detectable eye is extremely rare.

General measures.Often requiring urgent assistance - transfusion of blood components, the elimination of water-electrolyte disorders, inhalation of oxygen, intubation, mechanical ventilation, peritoneal dialysis and

hemodialysis.

Immunosuppressive therapy: plasmapheresis is carried out, at the same time it is prescribed predniozon cyclophosphamide.Plasmapheresis removes antibodies from the serum, and corticosteroids and immunosuppressants inhibit the production of these antibodies.After a few days or weeks after starting treatment and return to normal X-ray picture of lung function.CKD can progress even during treatment.Monotherapy with corticosteroids is effective for pulmonary hemorrhage, but has no effect on the course of glomerulonephritis.

the treatment of pulmonary hemosiderosis and Goodpasture's syndrome used hormonal and immunosuppressive drugs.In severe renal manifestations selection methods are dialysis or a kidney removal, followed by transplantation of the donor organ.In connection with anemia are recommended iron supplements, blood transfusion.

forecast.If untreated, 75% of patients die from chronic renal failure, 25% - from pulmonary hemorrhage.The same applies to improper treatment regimens: the use of immunosuppressive drugs in low doses or short course.However, the early holding of plasmapheresis and long-term treatment with prednisone and cyclophosphamide significantly improved the condition of patients and reduce the number of mortality.