Goodpasture's syndrome

Goodpasture's syndrome is a rare condition is mediated by anti-GBM antibody. It presents with recurrent haemoptysis and a severe progressive proliferative, often crescentic, glomerulonephritis.

There is a strong association with HLA-DR2. Lung haemorrhage, which occurs more commonly in cigarette smokers, responds to repeated plasma exchange (which removes the anti-GBM antibody) combined with immunosuppressive therapy with cytotoxic drugs and corticosteroids. The effect of this treatment upon the glomerulonephritis is less clear-cut; when oliguria occurs or serum creatinine rises above 600-700 μmol/L, renal failure is almost always irreversible.

There is an inherited predisposition for Goodpasture's syndrome and researchers believe that the gene or genes involved affect the way the immune system reacts to certain invaders, increasing the likelihood mistaken identity in the lungs and kidneys.

Cessation of pulmonary haemorrhage has been described after bilateral nephrectomy. Renal transplantation has been used and although there are IgG deposits in the graft it does not appear to damage the kidney. Most centres still like to wait 6 to 12 months before transplantation.

Without early treatment, permanent renal failure is the rule.