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.