Goodpasture's Syndrome

Goodpasture's Syndrome

Goodpasture’s syndrome (also known as Goodpasture’s disease and anti-glomerular basement antibody disease) is a rare autoimmune disease in which antibodies attack the lungs and kidneys, leading to bleeding from the lungs and to kidney failure. It may quickly result in permanent lung and kidney damage, often leading to death. It is treated with immunosuppressant drugs such as corticosteroids and cyclophosphamide, and with plasmapheresis, in which the antibodies are removed from the blood.

The specific target of the immune attack is the GBM antigen, which is found in the lungs and kidneys. The antigen is a component of the non-collagenous 1 (NC1) domain of the alpha-3 chain of type IV collagen in the glomerular basement membrane.

Goodpasture's syndrome is a type II hypersensitivity-like reaction.

The disease was first reported by the American pathologist Ernest Goodpasture of Vanderbilt University, in 1919.