Abstract
Background
Nephrotic syndrome relapse within 6 months is a known risk factor for steroid-dependent nephrotic syndrome/frequently relapsing nephrotic syndrome (SDNS/FRNS), but the risk of early development of SDNS/FRNS and initiation of immunosuppression therapy remains unknown.
Methods
Patients with childhood-onset idiopathic nephrotic syndrome who had the first relapse within 6 months were enrolled. We analyzed the relationship between the time of the first relapse or the time of initial remission and incidence of SDNS/FRNS or initiation of immunosuppression therapy.
Results
Forty-five patients were enrolled. Twenty out of 23 patients (87%) with the first relapse within 30 days after discontinuing initial steroid therapy experienced a second relapse within 30 days after discontinuing steroid therapy. Additionally, most patients in this group (96%) experienced a second relapse within 6 months after the onset and were diagnosed as SDNS/FRNS at this time. In this group, the incidence of SDNS/FRNS development within 6 months was 96%. In contrast, the incidence of SDNS/FRNS development within 6 months was 18% in patients with the first relapse more than 30 days after steroid discontinuation. The incidence of initiation of immunosuppressive agents within 6 months was 83% in the former group and 14% in the latter group.
Conclusions
Most patients with the first relapse within 30 days after discontinuing steroid therapy developed SDNS/FRNS and were administered immunosuppressive agents within 6 months. Thus, it might be reasonable to start immunosuppression therapy in this group without waiting for the second relapse.
Graphical abstract
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Data availability
The data supporting the findings of this study are available from the corresponding author upon reasonable request.
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All authors were physicians who treated the patients in this study. YI conducted the study, collected the clinical and laboratory data, and wrote the manuscript. KN, KO, TK, MA, MS, and MO reviewed and edited the manuscript. KK supervised the work and revised the manuscript. All authors read and approved the final manuscript.
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This study was approved by the Ethics Committee of the National Center for Child Health and Development (approval no. 2022–210). It was conducted according to the principles of the Declaration of Helsinki and the ethics guidelines of the Japanese Ministry of Health, Labor and Welfare.
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Due to the retrospective nature of this study, informed consent for participation was waived.
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Due to the retrospective nature of this study, informed consent to publish was waived.
Competing interests
Koichi Kamei has obtained research funding from the Public Foundation of Vaccination Research Center, the Terumo Foundation for Life Sciences and Arts, and the Taiju Life Social Welfare Foundation; donations from Chugai Pharmaceutical Co. Ltd., Astellas Pharma Inc., Ono Pharmaceutical Co. Ltd., Teijin Pharma Ltd., Shionogi Co. Ltd., and Otsuka Pharmaceutical Co. Ltd.; and lecture fees from Tanabe Mitsubishi Pharma, Baxter Ltd., and Zenyaku Kogyo Co. Ltd. All other authors have no potential conflicts of interest to disclose.
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Inoki, Y., Nishi, K., Osaka, K. et al. Association between the time of initial relapse and subsequent relapses in patients with childhood-onset idiopathic nephrotic syndrome. Pediatr Nephrol (2024). https://doi.org/10.1007/s00467-024-06286-9
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DOI: https://doi.org/10.1007/s00467-024-06286-9