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Adult survivors of childhood-onset steroid-dependent and steroid-resistant nephrotic syndrome treated with cyclosporine: a long-term single-center experience

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Abstract

Background

Although evidence has confirmed that cyclosporine (CS) is efficacious against childhood-onset steroid-dependent and steroid-resistant nephrotic syndrome (SD/SRNS), some patients may continue to relapse during adulthood. However, predictive factors for adult active disease and kidney complications, such as chronic kidney disease (CKD) and hypertension, in this cohort remain unknown.

Methods

We conducted a retrospective study on the long-term outcomes of 81 young adults with childhood-onset SD/SRNS treated with CS. The primary endpoint was the probability of active disease into adulthood. The secondary endpoint was the probability of developing kidney complications.

Results

At the last follow-up (median age, 23.2 years; median disease duration, 15.8 years), 44 adult patients (54%) continued to have active disease, whereas 16 patients developed CKD or hypertension, respectively. The proportion of patients developing kidney complications was similar between the active disease and long-term remission groups. Young age at NS onset and history of relapse during the initial CS (median, 31 months) were independent predictive factors for active disease. Acute kidney injury at NS onset, focal segmental glomerulosclerosis, and irreversible CS nephrotoxicity were identified as risk factors for the development of CKD, whereas older age was identified as a risk factor for the development of CKD and hypertension.

Conclusions

More than 50% of adult survivors treated with CS continued to have active disease, and each 20% developed CKD or hypertension. A long-term follow-up is necessary for patients with SD/SRNS to identify the development of kidney complications later in adulthood that can be attributed to prior disease and CS treatment in childhood, irrespective of disease activity.

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Data availability

The datasets of the current study are available from the corresponding author on reasonable request.

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Acknowledgements

The authors would like to thank Maruzen-Yushodo Co., Ltd. for the English language editing.

Funding

SF has received grants-in-aid for research from Saitama Children’s Medical Center.

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Authors and Affiliations

  1. Division of Nephrology, Saitama Children’s Medical Center, 1-2 Shintoshin, Chuo-Ku, Saitama City, Saitama, 330-8777, Japan

    Yoichi Takemasa, Shuichiro Fujinaga & Koji Sakuraya

  2. Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan

    Yoichi Takemasa & Daishi Hirano

  3. Department of Pediatrics, Faculty of Medicine, Juntendo University, Tokyo, Japan

    Mayu Nakagawa

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  1. Yoichi Takemasa
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Contributions

YT and SF designed the study, drafted the manuscript, and were responsible for the data integrity and analysis results. DH provided statistical support. All authors critically reviewed and approved the final manuscript.

Corresponding author

Correspondence to Shuichiro Fujinaga.

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Ethical approval

All procedures performed in this study involving human participants were in accordance with the ethical standards set by the ethics committee of Saitama Children’s Medical Center in which the study was conducted, and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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The Institutional Review Board waived the requirement for informed consent due to the retrospective nature of this study. The manuscript is available on a preprint server.

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The authors declare no competing interests.

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Takemasa, Y., Fujinaga, S., Nakagawa, M. et al. Adult survivors of childhood-onset steroid-dependent and steroid-resistant nephrotic syndrome treated with cyclosporine: a long-term single-center experience. Pediatr Nephrol 39, 473–482 (2024). https://doi.org/10.1007/s00467-023-06108-4

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