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Corticosteroid Sparing Agents in Frequent Relapsing and Steroid Dependent Nephrotic Syndrome in Children: A Single Center Retrospective Study

Bashair Alabbasi

Background: Children with Frequently Relapsing Nephrotic Syndrome (FRNS) or Steroid-Dependent Nephrotic Syndrome (SDNS) may be prescribed non-corticosteroid immunosuppressive agents whenever there is a failure to maintain remission with low-dose alternate-day prednisone and/or significant adverse effects of prednisone develop. A wide variety of immunosuppressive agents have been used in these patients to reduce the number of relapses and maintain remission.

Objectives: To evaluate the outcome of steroid sparing agents in the management of FRNS and SDNS in children with nephrotic syndrome.

Patients and methods: A retrospective study was conducted on all Steroid-Sensitive Nephrotic Syndrome (SSNS) children (1-11 years) who received any type of second line agents (e.g. CNI, MMF, cyclophosphamide, and Rituximab) over a period of 9 years from January 2010 to January 2019 in pediatric nephrology unit in prince sultan military medical city, Riyadh.

Results: The study included 24 patients. Their age at diagnosis ranged between 1 and 11 years with a mean of 3.8 years and standard deviation of ( ± ) 2.6 years. During the first year of steroid therapy, relapse occurred among 87% of patients; of them, the number of relapses being 4 or more in 21.7%. Regarding indication for the second line of treatment, SDNS was the most frequent reported (60.9%), followed by FRNS (30.4%). Concerning agents used in the second line, Mycophenolate Mofetil (MMF) ranked first (58.4%), followed by Cyclophosphamide (33.3%). Number of relapses after starting steroid sparing agent was more than once among 41.7% of patients. Duration of remission after starting steroid sparing agent ranged between 2 and 72 months (14 ± 14.1). Overall response to the second line of treatment was observed among majority of patients (91.7%). Renal biopsy was performed in 45.8% of patients. Concerning side effects of steroid sparing agents, electrolytes disturbances and hypertension were reported by two (8.3%) and one (4.2%) patients respectively. Duration of remission was significantly longer among patients treated with cyclosporine (48 ± 33.9 months) compared to other lines of treatment, p<0.001. On the other hand, hypertension was only reported among patients treated with cyclosporine, p=0.003.

Conclusion: The overall response of children with SDNS and FRNS to the second line agents was significant, with favorable longer remission free period with Cyclosporine use with no major side effects. Our results affected by the retrospective design of the study, as well as the small sample size. Therefore larger scale study with prospective design is highly encouraged.