Immunosuppressive therapy in children with steroid-resistant, frequently-relapsing, and steroid-dependent idiopathic nephrotic syndrome: a single center experience

INTRODUCTION: Immunosuppressive agents are recommended for the management of children with steroid-resistant (SRNS), frequently-relapsing (FRNS), and steroid-dependent idiopathic nephrotic syndrome (SDNS). This study evaluated the efficacy of immunosuppressive agents in these cases. METHODS: This is a retrospective analysis of the records of 130 pediatric cases recruited from a tertiary-care center over a period of two years. They were divided into two groups: 51 patients with SRNS (Group I) and 79 cases with SDNS and FRNS (Group II). They were treated with immunosuppressive agents in addition to steroids, either as double- or triple-combination therapy. Complete or partial remission was considered a good response. RESULTS: In group I, the proportions of good response to cyclophosphamide, cyclosporine A, and mycophenolate mofetil were 48.6, 60, and 80%, respectively (p = 0.162). In group II, the resistance rate was significantly higher with levamisole than with cyclophosphamide and azathioprine (p = 0.046). Leukopenia was reported infrequently after the administration of cyclophosphamide or azathioprine. The most serious adverse reaction was to cyclosporine A, which induced nephrotoxicity (6.4%), while no adverse effects related to levamisole were reported. Histopathological diagnoses were available in only 39 patients. CONCLUSION: The high potency of cyclosporine with steroids recommends its use in patients with idiopathic SRNS with a normal glomerular filtration rate. Its efficacy is augmented when combined with mycophenolate mofetil. Cyclophosphamide, orally or as intravenous boluses, together with alternate-day steroids, could be a good option outside the peripubertal age. The outcomes of FRNS and SDNS could be improved by encouraging compliance with the use of levamisole.