0A-S6-3

THERAPEUTIC OPTIONS IN STEROID-RESISTANT IDIOPATHIC NEPHROTIC SYNDROME

Patrick Niaudet, Hopital Necker-Enfants Malades, Paris, France

Steroid therapy should be withdrawn when this treatment has no effect. There is no data showing a beneficial effect of alkylating agents in steroid resistant patients with focal and segmental glomerular sclerosis (FSGS). Partial or complete remissions were reported in 20% of cases following a course of cyclophosphamide, which is close to the remission rate of patients receiving steroid therapy alone. A combined treatment with vincristine, cyclophosphamide and prednisolone to patients with steroid resistant nephrotic syndrome with FSGS induced lasting remission in 7 of 21 patients, starting 6 months to 3 years after initiation of treatment. Mendoza et al reported that combination of prolonged methylprednisolone pulse therapy with an alkylating agent was effective in children with steroid resistant FSGS. Cade et al reported a complete remission in 13 adult patients with steroid-resistant idiopathic nephrotic syndrome. Other studies failed to confirm these results

The French Society of Pediatric Nephrology reported on an open study involving sixty-one children with steroid resistant nephrosis who were treated with cyclosporine in combination with prednisone. Complete remission was observed in 44% of the children and none progressed to end stage renal failure. Thirty children failed to respond to the treatment and 43% of them progressed to renal failure.

The major problem of patients who progress to end-stage renal failure and who undergo renal transplantation is the risk of recurrence which occurs in 25% of cases. IV cyclosporine may be efficient in some cases. Plasmapheresis have been reported to induce remissions.