Goodpasture's Syndrome: Recovery from Severe Pulmonary Hemorrhage After Bilateral Nephrectomy

Abstract

A case of Goodpasture's syndrome is presented wherein life-threatening pulmonary hemorrhage, refractory to immunosuppressive drug therapy, rapidly resolved after bilateral nephrectomy. This experience, although not unprecedented, is reported here for the first time in a patient not in end-stage renal failure. During the 12 months that have elapsed since nephrectomy, there has been no recurrence of the patient's original disease; circulating antiglomerular basement membrane antibodies, present before nephrectomy, have become undetectable; and the patient has received a related living donor renal allograft. Electron microscopic studies of this patient's renal tissue showed effacement of the lamina rara interna, a previously unreported observation, and virus-like particles within glomerular endothelial cells. Based on the evidence for the immunopathogenesis of Goodpasture's syndrome and recent experiments with animal models for this disease, we offer an explanation of how nephrectomy may reverse pulmonary hemorrhage in Goodpasture's syndrome.