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Glucocorticoid discontinuation in pediatric-onset systemic lupus erythematosus: a single-center experience

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Abstract

Background

Glucocorticoid discontinuation, a challenge in systemic lupus erythematosus (SLE), might be achievable with the advent of new therapeutic options.

Methods

This single-center study included 31 children with newly diagnosed pediatric SLE between 2002 and 2021, after the exclusion of patients who were followed for less than 1 year after treatment initiation and those lost to follow-up. Patient characteristics, clinical course including flares, treatment, glucocorticoid discontinuation, and outcomes were retrospectively analyzed.

Results

Glucocorticoids could be discontinued in 19 (61%) patients during a median observation period of 105.5 (range, 17–221) months. Of these, 5 (26%), 12 (63%), and 18 (95%) patients could discontinue glucocorticoids in 3, 5, and 10 years from treatment initiation, respectively. Additionally, 18 of the 19 patients did not experience flares after glucocorticoid discontinuation during a median duration of 37.2 (7.2–106.8) months. Three of the nineteen patients achieved drug-free remission. At last follow-up, all patients achieved low disease activity with or without glucocorticoids and 19, 8, and 1 patient were receiving mycophenolate mofetil (MMF), MMF plus tacrolimus, and MMF plus ciclosporin A, respectively. Flares were observed in 15 patients during the observation period. MMF as initial immunosuppressant (P = 0.01) and shorter interval between therapy initiation and achieving maintenance prednisolone dose of 0.1–0.15 mg/kg/day (P = 0.001) were associated with significantly reduced flare risk. Femoral head necrosis was observed in two patients.

Conclusion

Despite the small sample size, these results support glucocorticoid discontinuation as a therapeutic target in pediatric SLE.

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Acknowledgements

The authors would like to thank Drs. Kentaro Ogata and Kentaro Matsuoka for their academic contribution and pathological diagnosis. The authors would also like to thank Mr. James R. Valera for his assistance with editing of the manuscript.

Author information

Authors and Affiliations

  1. Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan

    Kentaro Nishi, Masao Ogura, Sho Ishiwa, Toru Kanamori, Mika Okutsu, Shunsuke Yokota, Taishi Nada, Mai Sato, Koichi Kamei, Kenji Ishikura & Shuichi Ito

  2. Department of Pediatrics, Oita University, Faculty of Medicine, Oita, Japan

    Sho Ishiwa

  3. Department of Pediatrics, Tokyo Medical and Dental University, Tokyo, Japan

    Toru Kanamori & Mika Okutsu

  4. Department of Pediatrics, Kitasato University School of Medicine, Kanagawa, Japan

    Kenji Ishikura

  5. Department of Pediatrics, Yokohama City University Graduate School of Medicine, 3-9, Fukuura, Kanazawa-ku, Yokohama, Kanagawa, 236-0004, Japan

    Shuichi Ito

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  1. Kentaro Nishi
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Contributions

KN prepared the first draft of the manuscript and performed data collection and analysis. MOg, TK, SIs, MOk, SY, TN, and MS edited and reviewed the manuscript. SIt supervised and revised the manuscript. KI and KK revised and oversaw the work. All authors contributed to the study conception and design and approved the final manuscript.

Corresponding author

Correspondence to Shuichi Ito.

Ethics declarations

Ethics approval

This study (No. 1868) was approved by the Ethics Committee of the National Center for Child Health and Development.

Consent to participant

Informed consent for participating in this study was not required in accordance with the Declaration of Helsinki and the Ethical Guidelines for Medical and Health Research Involving Human Subjects of the Ministry of Health, Labor, and Welfare.

Consent for publication

Consent for publication was waived in accordance with the guidelines.

Conflict of interest

Koichi Kamei has received research funding from the Terumo Foundation for Life Sciences and Arts, Public Foundation of Vaccination Research Center, and Taiju Life Social Welfare Foundation and donations from Ono Pharmaceutical Co., Ltd. Kenji Ishikura has received lecture fees from Asahi Kasei Pharma, Chugai Pharmaceutical Co., Ltd, Zenyaku Kogyo Co., Ltd, and Novartis Pharma K.K. and grants from Asahi Kasei Pharma, Chugai Pharmaceutical Co., Ltd., Novartis Pharma, and Zenyaku Kogyo Co., Ltd. Shuichi Ito has received honoraria and research funding from Asahi Kasei Pharma, Chugai Pharmaceutical Co., Ltd, Zenyaku Kogyo Co., Ltd, and Astellas PHARMA Co., Ltd. Other authors have no potential conflicts of interest to disclose.

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Cite this article

Nishi, K., Ogura, M., Ishiwa, S. et al. Glucocorticoid discontinuation in pediatric-onset systemic lupus erythematosus: a single-center experience. Pediatr Nephrol 37, 2131–2139 (2022). https://doi.org/10.1007/s00467-021-05350-y

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  • DOI: https://doi.org/10.1007/s00467-021-05350-y

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