Goodpasture syndrome

Goodpasture syndrome

Goodpasture’s syndrome is an autoimmune disease characterized by pulmonary hemorrhage associated with severe progressive glomerulonephritis. AT to type IV collagen with a-3 chain (Goodpasture Ag) of the basement membrane of the renal glomeruli circulate in the blood.


    • kidneys
    • Epithelial cell crescents
    • Shrinkage of the renal glomeruli
    • Interstitial inflammatory exudate
    • Lungs
    • Intraalveolar hemorrhages
    • Macrophages loaded with hemosiderin
    • Fibrosis of the interalveolar septa.

Clinical picture

    • Lung damage (ahead of kidney damage) – repeated pulmonary bleeding with the development of respiratory failure
    • Rapidly progressive glomerulonephritis with renal failure
    • Flu-like syndrome: fever, myalgia, arthralgia, weakness.


    • IDA
    • Hematuria, proteinuria
    • AT against Ag of the basal membrane of the glomeruli of the kidneys
    • Renal biopsy – deposits of immunoglobulins and complement in the glomerular basement membrane
    • X-ray of the chest – volatile asymmetric cloud-like infiltrates; lung destruction is uncommon.


    • Diet number 7a.
    • Glucocorticoids
    • Pulse therapy with methylprednisolone 30 mg/kg/day IV drip for 20-30 minutes for 3 consecutive days
    • Prednisolone can be taken in an alternating regimen – 48 hours after pulse therapy at a dose of 2 mg / kg orally for 15 days, 1.75 mg / kg for 1 month, 1.5 mg / kg for 3 months, then each dose is prescribed for 6 months (1.25-1.0-0.75-0.5-0.25 mg/kg), then each dose is prescribed for 12 months (0.125-0.0625 mg/kg). Notes
    • Diuretics are not recommended 3 hours before and 24 hours after pulse therapy.
    • Patients over 60 years of age should reduce the dose by 25%.
    • Cyclophosphamide nbsp; – against the background of glucocorticoids for 3 months, respectively, at doses of 2.0-1.5-1.0-0.5 mg / kg. Notes: the dose of the product should be reduced by 50% when the glomerular filtration rate drops to 10 ml / min; it is necessary to control the number of leukocytes and blood platelets.
    • Antiplatelet agents – dipyridamole 75 mg 3 times a day.
    • Plasma exchange of 4 liters every other day with a replacement of 5% serum albumin against the background of immunosuppressive therapy.
    • Hemodialysis in the development of renal failure.
    • Kidney transplantation in the terminal stage of chronic renal failure.

Synonym. Hereditary pulmonary-renal syndrome See also Wegener’s granulomatosis, Systemic lupus erythematosus, Pulmonary hemosiderosis, Respiratory failure, Chronic renal failure ICD M31.0 Hypersensitivity angiitis MIM. 233450 Goodpasture’s syndrome



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