Goodpasture’s syndrome is characterized by –
Correct Answer: Necrotising hemorrhagic interstitial pneumonitis
Description: Goodpasture syndrome
Goodpasture syndrome is a rare condition characterized by the rapid destruction of the kidney and diffuse pulmonary hemorrhage.
It is an autoimmune disease characterized by the presence of circulating autoantibodies targeted against basement membrane of lung and kidney.
These antibodies are directed against the noncollagenous domain of the α -3 chains of type IV collagen (collagen of basement membrane).
The antibodies initiate an inflammatory destruction of the basement membrane in kidney glomeruli and lung alveoli.
In Goodpasture syndrome, the immune reaction is type II hypersensitivity.
Morphological changes
I. Lung
The lungs are heavy, with areas of red-brown consolidation.
There is focal necrosis of alveolar walls associated with intraalveolar hemorrhages.
Alveoli contain hemosiderin-laden macrophages.
Linear deposits of immunoglobulins along the basement membranes of the septal walls.
II. Kidney
Diffuse proliferative rapidly progressive glomerulonephritis.
Focal necrotizing lesion and crescents in >50% of glomeruli.
Linear deposits of immunoglobulins and complement along the glomerular basement membrane.
Clinical manifestations
Occur typically in young males.
Most cases begin clinically with respiratory symptoms, principally hemoptysis.
Soon, manifestations of glomerulonephritis appear and typically present as nephritic syndrome → hematuria, nephritic urinary sediment, subnephrotic proteinuria, rapidly progressive renal failure.
The common cause of death is renal failure.
Category:
Pathology
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