Abstract
Background
Cases of Henoch–Schönlein purpura nephritis (HSPN) with moderate severity were demonstrated to achieve good prognosis after treatment with renin-angiotensin system (RAS) inhibitors. However, some patients required additional treatment for recurrence after remission. This study aimed to clarify the effect of RAS inhibitors in HSPN cases with moderate severity, including the proportion of cases with recurrence and their response to additional treatment.
Methods
Among 126 patients diagnosed with HSPN between 1996 and 2019, 71 patients with clinicopathologically diagnosed HSPN of moderate severity, defined as ISKDC grade II–IIIa and serum albumin ≥ 2.5 g/dL, were investigated.
Results
Proteinuria became negative after RAS inhibitor treatment alone in all 71 cases. However, 16 (22.5%) had recurrence. Eleven recurrent cases achieved negative proteinuria again following additional treatment. At the last follow-up (median 46.5 months; IQR, 23.2–98.2), 5 patients had persistent mild proteinuria; no patients had estimated glomerular filtration rate < 90 mL/min/1.73 m2. The pathological findings in all recurrent cases were ISKDC grade IIIa. The 16 recurrent cases had significantly higher proportions of glomeruli with global/segmental sclerosis (25.0 vs. 0%, P < 0.001) and tubular atrophy/interstitial fibrosis (37.5 vs. 12.7%, P =0.0 24) than 55 cases without recurrence.
Conclusions
Japanese childhood HSPN cases with moderate severity had good outcomes without need for corticosteroids or immunosuppressants, when prescribed RAS inhibitor treatment. Even in recurrent cases, abnormal proteinuria was transient, and prognosis was excellent.
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Data from this study can be obtained from the corresponding author on reasonable request.
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Acknowledgements
We thank all of the study patients. We also extend our sincere thanks to the participating doctors for providing clinical information. Finally, we thank Alison Sherwin, PhD, from Edanz (https://jp.edanz.com/ac), for editing a draft of this manuscript.
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SN, TH, AK, YA, SI, NS, CN, TY, TN, HK, RT, YS, JF, NK, and SI managed the patients and conceived the study; SN, TN, and TH drafted the manuscript; KoN, NY, KI, and KaN reviewed the manuscript. All the authors have read and approved the final manuscript.
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This study involving human participants was conducted in accordance with the Declaration of Helsinki. Ethics approval was obtained from the Ethics Review Committee of Kobe University Graduate School of Medicine.
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Informed consent for kidney biopsy was obtained and published consent requirements were not necessary because the study was retrospective.
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Nagai, S., Horinouchi, T., Ninchoji, T. et al. Use of renin-angiotensin system inhibitors as initial therapy in children with Henoch–Schönlein purpura nephritis of moderate severity. Pediatr Nephrol 37, 1845–1853 (2022). https://doi.org/10.1007/s00467-021-05395-z
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DOI: https://doi.org/10.1007/s00467-021-05395-z