Cyclosporine a therapy in idiopathic nephrotic syndrome : a study of 14 cases

Abstract EN

Objective : Cyclosporine A therapy has a well established use in patients with steroiddependent and steroidresistant nephrotic syndrome.

This study was conducted at King Hussein Medical Center describing our experience in the use of cyclosporine A in such patients.

Methods: A total number of 14 patients with steroid dependent (n = 10) and steroid resistant nephrotic syndrome (n = 4) were included in the study.

Age ranged from 4.5 to 14 years with a mean of 9.5 ± 4.9 years.

All patients with steroid dependent nephrotic syndrome received cyclophosphamide therapy prior to cyclosporine A and had at least one steroidal side effect Kidney function tests were normal for all patients.

Kidney biopsy was done for all patients prior to the introduction of cyclosporine A therapy.

Cyclosporine A was given at a dose of 4-6 mg/kg/day orally in two divided doses and adjusted to maintain a mean blood trough level between 100 and 150,ng/ml.

The mean duration of therapy was 13.0 ± 8.4 months.

Results: Based on kidney biopsy results 9 patients of the steroid dependent group had minimal change nephrotic syndrome and one patient had focal segmental glomerulosclerosis.

Of the steroid resistant group one patient had minimal change disease, two patients had focal segmental glomerulosclerosis and one patient had mesangiocapillary glomerulonephritis.

All patients who had minimal change nephrotic syndrome responded initially and achieved remission on cyclosporine A alone within one month of starting treatment Four patients relapsed after 4-6 months, Jiowever, they responded when low dose steroid was Reintroduced and continued in remission.

Two patients relapsed after stopping cyclosporine A, which lasted for 24 months.

Two patients with focal segmental glomerulosclerosis responded (one steroid dependent and one steroid resistant nephrotic syndrome).

One patient with focal segmental glomerulosclerosis and one with mesangiocapillary glomerulosclerosis showed no response at all and Cyclosporine A was discontinued after completing 8 weeks of treatment Conclusion : Cyclosporine A therapy seems to be effective in inducing remission and decreasing steroid requirements in children with minimal change nephrotic syndrome.

Its use in focal segmental glomerulosclerosis, though not as effective, shows promising results.