Goodpasture Syndrome
Goodpasture syndrome - a systemic capillary with a predominant lesion of the lungs and kidneys by the type of hemorrhagic pneumonitis and glomerulonephritis. Men are more often ill at the age of 20 - 30 years.
The etiology is unknown, but there is a connection with a viral and bacterial infection, hypothermia. The first description of the disease was made during the flu epidemic in 1919. Autoimmune pathogenesis is discussed, since circulating and fixed antibodies to the basal membranes of the kidneys cross-reacting with the basal membrane antigens are found.
Symptoms, course. Start acute with high fever, hemoptysis or pulmonary hemorrhage, shortness of breath. With auscultation, an abundance of voiced wet wheezing in the middle and lower sections of the lungs, radiologically - multiple focal or draining blackouts in both pulmonary fields. Heavy, progressive glomerulonephritis develops almost simultaneously, rapidly leading to renal failure. Repeated hemoptysis and hematuria lead, as a rule, to anemia, aggravated with kidney failure. In the laboratory study: anemia, leukocytes and elevated ESR. A characteristic immunological sign of the disease are antibodies to the basal membranes of the kidney. The prognosis is usually unfavorable - death occurs in the next 6 months - 1 year from the onset of the disease with pulmonary-cardiac or renal insufficiency.
Treatment. Corticosteroids in large doses (prednisolone up to 100 mg / day) in combination with cytotoxic drugs (azathioprine 150 - 200 mg / day) provided early treatment can slow the progression of the disease. The successful use of plasmapheresis in combination with immunosuppressive therapy is described.
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