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

Abstract

INTRODUCTION Immunosuppressive agents are recommended for the management of children with steroid-resistant, frequently-relapsing, and steroid-dependent idiopathic nephrotic syndrome, i.e., SRNS, FRNS, and 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, i.e., 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 were reported to be related to levamisole. Histopathological diagnoses were available in only 39 patients. CONCLUSION The high potency of cyclosporine with steroids makes it useful 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 with FRNS and SDNS could be improved by encouraging compliance with the use of levamisole.