Antibody Mediated

Anti-Glomerular Basement Membrane Disease

Goodpasture's syndrome is a clinical constellation

of glomerulonephritis and pulmonary hemorrhage, mediated by an anti-glomerular basement membrane antibody which also reacts with the basement membrane of pulmonary capillaries. Up to one-third of patients with rapidly progressive glomerulonephritis and anti-GBM antibodies, do not have detectable evidence of pulmonary involvement (anti-glomerular basement membrane disease). 

Since the antibody response is directed toward a normal antigen present in the GBM, Goodpasture's syndrome and anti-GBM disease are classic examples of autoimmune disorders. 

Clinical Course

Goodpasture's syndrome may occur at any time, but peaks in the spring and summer months are common. It frequently begins with a flu-like illness with evidence of pulmonary compromise. Progressive dyspnea, hemoptysis and occasionally ventilatory failure occur. Urinalysis reveals hematuria with red blood cell casts and variable proteinuria. 

Patients with Goodpasture's syndrome require immediate institution of plasma exchange and immunosuppressive therapy. 

Prompt institution of therapy usually results in significant improvement in renal function. Patients with an initial serum creatinine less than 7 mg/dl and fewer than 50% crescents have a greater liklihood of responding favorably to therapy.