Lupus nephritis with nephrotic syndrome is one of the most serious complications of systemic lupus erythematosus. Six female patients with systemic lupus and nephrotic syndrome, refractory to immunosuppressive drug therapy, received 15-20 exchange plasmaphereses. One patient, treated concurrently with high-dose steroids, showed temporary improvement, and five patients, treated concurrently with steroids and either cyclophosphamide or azathioprine, had long-term remissions. Plasmapheresis is a promising therapeutic modality in cases of refractory lupus nephritis with nephrotic syndrome.