Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome

Abstract

We conducted a prospective, multicenter trial to evaluate the efficacy and safety of a 12-month course of cyclosporine in children with steroid-resistant nephrotic syndrome (SRNS). Thirty-five patients were enrolled, of whom 28 had minimal change or diffuse mesangial proliferation (MC/DMP), and seven had focal segmental glomerulosclerosis (FSGS). All patients received cyclosporine and prednisolone; patients with FSGS additionally received methylprednisolone pulse therapy (MPT). The dose of cyclosporine was adjusted to maintain a trough level of 120-150 ng/ml during the initial 3 months of treatment, followed by 80-100 ng/ml during months 4-12. The primary end point was the remission rate at month 12. Remission was achieved in 23 of 28 (82.1%) patients in the MC/DMP group and in six of the seven (85.7%) patients in the FSGS group. Follow-up renal biopsies were performed in 26 patients (nine at month 12, 17 at month 24), and cyclosporine-related nephrotoxicity was detected in one (3.8%). Major adverse events comprised severe bacterial infections (two patients) and posterior reversible encephalopathy syndrome (one patient). In conclusion, a high remission rate was achieved in our patient cohort using a combined cyclosporine/ prednisolone treatment regimen in children with SRNS who had MC/DMP and a combined cyclosporine/prednisolone plus MPT regimen in children who had FSGS.

Clinical Profile and Outcome of Steroid Resistant Nephrotic Syndrome in Children: An Eastern Indian Single Centre Study

“…MMF is a promising therapeutic alternative without nephrotoxicity [20]. But there is no single consensus regarding the indications of renal biopsy and treatment protocols in children with SRNS [13,14,15,16]. Optimal combinations of these agents with the least toxicity remain to be determined [12].…”

Glucocorticoid receptors expression and histopathological types in children with nephrotic syndrome

“…Also, in Saudi Arabia and Tunisie (countries comparable to Egypt), the incidence of FSGS is more important than MCD in steroid-resistant cases. 23,24 However, two studies from Japan and France reported a significantly higher incidence of MCD compared to FSGS in steroid-resistant NS. 11,24 The reasons for disparities in the frequency of MCD and FSGS are not entirely clear.…”

“…23,24 However, two studies from Japan and France reported a significantly higher incidence of MCD compared to FSGS in steroid-resistant NS. 11,24 The reasons for disparities in the frequency of MCD and FSGS are not entirely clear. They are probably explained by racial, genetic and environmental factors.…”

Consenso de tratamiento del síndrome nefrótico en la infancia

“…Estos cambios van desde la toxicidad aguda y la vacuolización isomérica de las células epiteliales tubulares hasta la fibrosis intersticial renal en un 80% de ellos cuando el tratamiento se prolonga por 4 años. [42][43][44][45][46] Los últimos resultados publicados aportan una remisión de la proteinuria en un 80% en pacientes corticorresistentes con esclerosis focal. [46][47][48] Otras recomendaciones para el tratamiento de pacientes que no responden a la ciclofosfamida son ácido micofenólico, tacrolimos y rituximab.…”