Long-term cyclosporin A treatment in adults with refractory nephrotic syndrome

Patients with refractory nephrotic syndrome (NS) are at risk of infections, thrombosis, renal failure, or inherent side effects of immunosuppressive therapy. In the present study we investigated the efficacy of cyclosporin A (Cy A) in treatment of adult patients with steroid-refractory NS. Fifteen patients were included, 13 of whom were steroid resistant. Initial renal histology showed minimal-change glomerulonephritis (MCGN) in 3 patients, focal segmental glomerulonephritis (FSGN) in 6 patients, and membranous glomerulonephritis (MGN) in 6 patients. Two steroid-dependent patients (one with MCGN and the other with FSGN) were included due to severe steroid side effects. Complete remission (CR) was achieved in those 2 patients, while in the steroid-resistant patients remission was only partial response (PR) and occurred in about half of the patients in each histological subgroup. In patients who responded to Cy A treatment, two attempts were made to taper off the drug, after 6 and 24 months. Unfortunately, both attempts were unsuccessful and NS relapsed. Except for hypertension in those with advanced renal insufficiency, the drug was well tolerated on long-term usage. A second kidney biopsy was carried out in patients in whom renal failure progressed despite Cy A therapy, and who did not show evidence of Cy A toxicity. These findings are in favor of Cy A for treatment of patients with refractory NS. Treatment with Cy A should be maintained for a minimum period of 3 months before considering its failure. The drug was found to be superior to steroid and conventional immunosuppressive drugs in treatment of steroid-refractory NS; however, the response was partial and was limited to only one-half of the patients in the different subgroups.