The use of steroid-sparing agents in steroid-sensitive nephrotic syndrome

Abstract

Childhood nephrotic syndrome (NS) is frequently characterized by a relapsing course. There is no uniform agreement about the precise stage at which a steroid-sparing agent should be introduced to control the disease. In order to evaluate the treatment strategies and outcome of steroid-sensitive NS over the last 2 decades, a retrospective notes review was undertaken in a cohort of children treated at Great Ormond Street Children's Hospital between 1980 and 2000. From a population of 863 children with NS referred, 509 had frequently relapsing or steroid-dependent disease and 261 children received at least one steroid-sparing agent. Cyclophosphamide was the first choice in 178 patients and in 114 no further steroid-sparing agent was needed. Levamisole was prescribed as the first steroid-sparing agent for 65 children and disease control was achieved in 30%. Cyclosporin A was prescribed in 61 children and sustained remission was induced in 69%. It is concluded that cyclophosphamide is a potent agent in inducing sustained remission in steroid-sensitive NS. Levamisole and cyclosporin A have emerged as attractive steroid-sparing agents. Complications and major side effects of treatment are infrequent but occasionally fatal.

Short courses of daily prednisolone during upper respiratory tract infections reduce relapse frequency in childhood nephrotic syndrome

“…The use of tapering dose regimens and maintaining prednisolone at a dose of 0.1-1mg per kg have proved effective in maintaining remission in those relapsing frequently [4]. Numerous steroid-sparing agents such as levamisole, cyclosporine A, cyclophosphamide, mycophenolate mofetil (MMF), chlorambucil and more recently rituximab, have been used with varying success rates in patients with significant steroid induced adverse effects [4,5,6].…”

Clinical Course of Steroid Sensitive Nephrotic Syndrome in Children: Outcome and Outlook

“…However, the longterm PRED treatment seriously increases the risk of AEs among these patients, which in turn motivates alteration of treatment. The steroid-sparing agents based treatment is the main approach for reducing relapse rate and thereby avoiding steroid-related AEs [3,11,[14][15][16]. During the last years, the alkylating agents in the case of NS relapse are not of first priority.…”

“…Nevertheless, CYC I.V. is usually recommended as an optional treatment to maintain remission in SSNS [7,11,[13][14][15][16][17][18][19][20][21][22]. In different studies, the percentage of relapse-free patients varies between 5 and 67% during 24 months, and between 17 and 65% during 60 months, respectively [5,18,[19][20][21][22][23][24].…”

Childhood nephrotic syndrome: steroid sensitive, steroid resistant and beyond

“…CSA was used in 81 children in order to control the disease and in 9 pubertal children it was prescribed as the first steroid sparing therapy. Chlorambucil was used in 16 children when other forms of steroid sparing therapy had failed 7 . Infection is a universal concern in children with NS especially when receiving cytotoxic or immunosuppressive therapy.…”