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Baseline characteristics and long-term outcomes of steroid-resistant nephrotic syndrome in children: impact of initial kidney histology

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Abstract

Background

Although many pediatric nephrologists consider focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) as separate clinical entities, whether the initial histology could affect clinical courses in children with steroid-resistant nephrotic syndrome (SRNS) suspected of having an immune-based etiology remains unknown, especially for long-term outcomes.

Methods

We retrospectively reviewed long-term outcomes (> 3 years; median follow-up, 9.1 years) of 21 children with initial SRNS (FSGS, N = 9; MCD, N = 12) who achieved complete remission with immunosuppressive agents, including cyclosporine.

Results

At NS onset, incidence of acute kidney injury (67% vs. 8%, P < 0.05) and proportion of patients with non-selective proteinuria (56% vs. 0%, P < 0.01) were significantly higher in the FSGS group than the MCD group. Furthermore, median days until complete remission after treatment was significantly longer in the FSGS group than the MCD group (116 days vs. 45 days, P < 0.001). Although subsequent biopsy histology of the 12 patients in the MCD group was still identical in all MCD, three of nine patients in the FSGS group were reclassified from FSGS to MCD at second biopsy. At last visit, all patients maintained complete remission, and none developed chronic kidney disease.

Conclusions

Initial presentation in the FSGS group was characterized by more severe clinical manifestations than the MCD group. If complete remission is achieved, FSGS and MCD in children with immune-mediated SRNS may constitute a single disease spectrum because the long-term outcomes are favorable, irrespective of initial histology.

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Acknowledgments

The authors wish to thank Dr. Hitohiko Murakami (Division of Pathology, Saitama Children’s Medical Center) for his great contributions to this study.

Funding

SF has received clinical research funding B at 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

    Yoshitaka Watanabe, Shuichiro Fujinaga, Shota Endo, Mayu Nakagawa & Koji Sakuraya

  2. Children’s Medical Center, Showa University Northern Yokohama Hospital, Yokohama, Japan

    Yoshitaka Watanabe

  3. Department of Pediatrics and adolescent medicine, Juntendo University, Tokyo, Japan

    Amane Endo

Authors
  1. Yoshitaka Watanabe
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  2. Shuichiro Fujinaga
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  3. Amane Endo
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  4. Shota Endo
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  5. Mayu Nakagawa
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  6. Koji Sakuraya
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Corresponding author

Correspondence to Shuichiro Fujinaga.

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The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the Institutional Research Committee and/or National Research Committee at which the study was conducted (approval number 2019-03-009) with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Watanabe, Y., Fujinaga, S., Endo, A. et al. Baseline characteristics and long-term outcomes of steroid-resistant nephrotic syndrome in children: impact of initial kidney histology. Pediatr Nephrol 35, 2377–2381 (2020). https://doi.org/10.1007/s00467-020-04760-8

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  • DOI: https://doi.org/10.1007/s00467-020-04760-8

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