CYCLOSPORINE IN THE THERAPY OF STEROID-RESISTANT IDIOPATHIC NEPHROTICSYNDROME

Steroid-resistant nephrotic syndrome with either minimal changes or fo cal and segmental glomerular sclerosis on initial biopsy is a severe c ondition as more than 50% of patients with the disease progress to end -stage renal failure within 10 years. We recently identified a distinc t form of idiopathic nephrotic syndrome with an autosomal recessive mo de of inheritance and were able to map the gene on the long arm of chr omosome 1. The absence of recurrence of the disease after renal transp lantation suggest a primary defect in a glomerular basement membrane p rotein. Several reports suggest that cyclosporine is effective in a pr oportion of patients with steroid-resistant idiopathic nephrotic syndr ome, particularly when used in combination with corticosteroids. There are also data suggesting that high doses of cyclosporine may be neces sary in patients with severe hypercholesterolemia. However, cyclospori ne treatment should be carefully monitored in view of the high risk of nephrotoxicity, as shown by the results of repeat renal biopsies. The beneficial role of cyclosporine in recurrent steroid-resistant nephro tic syndrome is still debated. Preliminary observations suggest that t he early use of intravenous cyclosporine may be effective in these pat ients.