Showing posts with label GOODPASTURES SYNDROME. Show all posts
Showing posts with label GOODPASTURES SYNDROME. Show all posts

GOODPASTURE'S SYNDROME

GOODPASTURE'S SYNDROME
Rare disease characterized by intra-alveolar hemorrhage and glomerulonephritis.
Etiology and Pathogenesis
  • A cytotoxic antibody against glomerular and alveolar basement membrane is responsible for the injury.
  • By activating complement, the antibody causes damage to glomerular and alveolar basement membrane.
  • A respiratory viral infection is believed to initiate production of the antibody.
  • Antigenic similarity between kidney and lung basement membrane accounts for the clinical picture.
Pathology
Clinical Features
  • Pulmonary complaints consist of hemoptysis and dyspnea.
  • Hematuria, proteinuria, red cell casts and renal failure are results of glomerulonephritis.
  • Iron deficiency anemia results from pulmonary hemorrhage.
  • Diffuse bilateral alveolar infiltrates in chest x-ray.
  • Hypoxemia and restrictive defect of PFT's.
  • Leniar deposition of IgG and complement along glomerular and alveolocapillary basement membranes, characteristic of type II reaction in lung and kidney biopsies.
  • EM
  • Antiglomerular basement membrane antibody can be detected in serum.
Therapy

  • Steroid in high doses controls pulmonary hemorrhage.
  • Nephrectomy with dialysis is necessary in some patients.
  • Combination of plasmapheresis and immunosuppressive therapy provides the best results.

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