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

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 patient...

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Veröffentlicht in:Pediatric nephrology (Berlin, West) West), 2022-09, Vol.37 (9), p.2131-2139
Hauptverfasser: Nishi, Kentaro, Ogura, Masao, Ishiwa, Sho, Kanamori, Toru, Okutsu, Mika, Yokota, Shunsuke, Nada, Taishi, Sato, Mai, Kamei, Koichi, Ishikura, Kenji, Ito, Shuichi
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Sprache:eng
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Zusammenfassung: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.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-021-05350-y