Anti-glomerular basement membrane glomerulonephritis:
An autoimmune disorder characterized by
small vessel vasculitis; in this version, we see linear IgG deposition along the basement membrane.
When basement membranes in the lungs are also involved, we call this
Goodpasture's syndrome.
In addition to nephritic syndrome, these patients also have hemorrhaging from the alveolar capillaries.
Timely diagnosis and treatment with plasma exchange and corticosteroids improve prognosis; otherwise, this disorder proceeds to rapidly progressing GN (crescentic GN).
GOODPASTURE'S SYNDROME
Goodpasture’s syndrome is an autoimmune disorder caused by antibodies that attack the basement membranes of
renal glomeruli and/or
respiratory alveoli; specifically, the antibodies attack type IV collagen in the tissues.
- In a histological sample of an affected glomerulus, we can see the characteristic crescent-shaped area formed by excessive fibrin and cellular material.
- In a sample of lung tissue, we highlight hemorrhaging in the inflamed alveoli.
- Goodpasture’s syndrome may be treated with corticosteroids and cyclophosphamide, or plasmapheresis, which removes the attacking antibodies.