Introduction:
Childhood onset nephrotic syndrome generally is steroid sensitive and resolves spontaneously on reaching puberty. However, 50% of patients have frequent relapses or become steroid dependent which requires prolonged exposure to steroids, and in turn, may require steroid sparing agents. There is insufficient evidence regarding the choice and timing of use of steroid sparing agents. Cyclophosphamide and Levamisole are recommended to be started after the patient has achieved remission with steroids. No such recommendation exists for Mycophenolate Mofetil and Calcineurin Inhibitors. Literature to date is lacking on the use of steroid-sparing agents before a child has achieved remission and whether it is a more effective approach to induce early remission and limit the number of relapses occurring in FRNS and SDNS cases.
Methods:
An investigator-initiated, prospective, open-label, randomized, two-arm parallel-group superiority trial comparing two different approaches in treating patients aged 1-14 years diagnosed with FRNS/SDNS presenting in relapse. In arm A, steroid-sparing agents were simultaneously started with steroids. In arm B, a steroid-sparing agent was added after the child had achieved remission.
Results:
The median age of the first attack of nephrotic syndrome in our cohort of FRNS/SDNS patients was 36 months. There was a male preponderance in patients with nephrotic syndrome. On kidney biopsy, minimal change disease (MCD) was the most common glomerular pathology seen. Null hypothesis was found to be true. There was no statistical difference between the two arms in achieving remission. Group A (prednisolone + steroid-sparing agent) reported fewer relapses which approached statistical significance (p = 0.081). There was no difference in the number of serious infections between the groups.
Conclusions:
Prednisolone plus steroid sparing agent is non-inferior to solo Prednisolone therapy in achieving remission in FRNS/SDNS patients with relapse, however, it may be beneficial in decreasing overall number of relapses.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.