[The treatment of children with idiopathic nephrotic syndrome using cyclosporin]
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Published on: November 8, 2015
R A Donckerwolcke1, J G Vande Walle
1Universiteitskliniek Wilhelmina Kinderziekenhuis, Utrecht.
Insights
Cyclosporine effectively induced remission in children with nephrotic syndrome, but relapses occurred after discontinuation. Severe nephrotoxicity limited its use, suggesting restriction to steroid-dependent cases with significant steroid toxicity.
Area of Science:
- Pediatric Nephrology
- Immunosuppressive Therapy
Context:
- Steroid-dependent and steroid-resistant nephrotic syndrome (NS) pose significant challenges in pediatric care.
- Current treatment strategies often involve corticosteroids, which can lead to toxicity and dependence.
Purpose:
- To evaluate the efficacy and safety of cyclosporine in children with steroid-dependent and steroid-resistant nephrotic syndrome.
- To assess remission rates, recurrence patterns, and long-term histopathological changes associated with cyclosporine treatment.
Summary:
- Cyclosporine, at 6 mg/kg/day targeting blood levels of 60-100 ng/ml, induced remission in 15 of 21 pediatric NS patients.
- Remission was sustained off prednisone in 9 patients, but 5 relapsed upon cyclosporine withdrawal.
- Nephrotoxicity was observed in 9 of 13 patients undergoing repeat biopsy, necessitating cyclosporine withdrawal in 4.
Impact:
- Cyclosporine demonstrates potential for inducing remission in difficult-to-treat pediatric nephrotic syndrome.
- Careful patient selection is crucial due to the risk of significant nephrotoxicity.
- Findings support restricting cyclosporine to steroid-dependent NS patients experiencing severe steroid-related toxicity.
Abstract:
We report our experience with cyclosporine in 14 children with steroid dependent and 7 children with steroid resistant nephrotic syndrome. Cyclosporine was started at a dose of 6 mg/kg/day and was adjusted to maintain a blood level between 60 and 100 ng/ml. 15 patients went into remission with a combined treatment of cyclosporine and prednisone; 9 of these did not have a recurrence following discontinuation of prednisone. In 7 out of these 9 patients cyclosporine was also discontinued, this led in 5 of these 7 patients to a recurrence. Seven out of 14 patients with minimal change nephropathy remained in remission following discontinuation of prednisone and in 4 others the dose of steroid required to maintain remission decreased. In only I out of 4 patients with focal segmental glomerulosclerosis was a remission obtained while in two patients with mesangial proliferation the urine remained protein free during cyclosporine treatment. In 13 patients a repeat biopsy was performed after one year or longer of cyclosporine treatment. Histopathological abnormalities related to cyclosporine were found in 9 patients. In 4 of these severe nephrotoxicity required withdrawal of cyclosporine. Treatment with cyclosporine should be restricted to steroid dependent patients with severe steroid toxicity.
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