1. Rovin BH, Adler SG, Barratt J, Bridoux F, Burdge KA, Chan
TM, et al. Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases. Kidney Int.
2021;100(4):753–79. https://doi.org/10.1016/j.kint.2021.05.015.
2. Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel
S, et al. IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic
syndrome. Pediatr Nephrol. 2023;38(3):877–919. https://doi.org/
10.1007/s00467-022-05739-3.
3. Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet.
2003;362(9384):629–39. https://d oi.o rg/1 0.1 016/S
0140-6 736(03)
14184-0.
4. Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr. Prognostic significance of the early course of minimal change nephrotic
syndrome: report of the International Study of Kidney Disease in
Children. J Am Soc Nephrol. 1997;8(5):769–76.
5. Short versus standard prednisone therapy for initial treatment of
idiopathic nephrotic syndrome in children. Arbeitsgemeinschaft
fur Padiatrische Nephrologie. Lancet. 1988;1(8582):380–3
6. Hodson EM, Wong SC, Willis NS, Craig JC. Interventions for idiopathic steroid-resistant nephrotic syndrome in children. Cochrane
Database Syst Rev. 2016;10:CD003594. https://doi.org/10.1002/
14651858.CD003594.pub5.
7. Shenoy M, Plant ND, Lewis MA, Bradbury MG, Lennon R,
Webb NJ. Intravenous methylprednisolone in idiopathic childhood nephrotic syndrome. Pediatr Nephrol. 2010;25(5):899–903.
https://doi.org/10.1007/s00467-009-1417-1.
8. Yorgin PD, Krasher J, Al-Uzri AY. Pulse methylprednisolone
treatment of idiopathic steroid-resistant nephrotic syndrome.
Pediatr Nephrol. 2001;16(3):245–50.
Clinical and Experimental Nephrology 9. Hamasaki Y, Yoshikawa N, Hattori S, Sasaki S, Iijima K,
Nakanishi K, et al. Cyclosporine and steroid therapy in
children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2009;24(11):2177–85. https://d oi.o rg/1 0.1 007/
s00467-009-1264-0.
10. Gipson DS, Chin H, Presler TP, Jennette C, Ferris ME, Massengill
S, et al. Differential risk of remission and ESRD in childhood
FSGS. Pediatr Nephrol. 2006;21(3):344–9. https://doi.org/10.
1007/s00467-005-2097-0.
11. Mori K, Honda M, Ikeda M. Efficacy of methylprednisolone pulse
therapy in steroid-resistant nephrotic syndrome. Pediatr Nephrol.
2004;19(11):1232–6. https://d oi.o rg/1 0.1 007/s 00467-0 04-1 584-z.
12. Hattori M, Chikamoto H, Akioka Y, Nakakura H, Ogino D,
Matsunaga A, et al. A combined low-density lipoprotein apheresis and prednisone therapy for steroid-resistant primary focal
segmental glomerulosclerosis in children. Am J Kidney Dis.
2003;42(6):1121–30.
13. Franke D, Zimmering M, Wolfish N, Ehrich JH, Filler G. Treatment of FSGS with plasma exchange and immunadsorption. Pediatr Nephrol. 2000;14(10–11):965–9.
14. Feld SM, Figueroa P, Savin V, Nast CC, Sharma R, Sharma M,
et al. Plasmapheresis in the treatment of steroid-resistant focal
segmental glomerulosclerosis in native kidneys. Am J Kidney Dis.
1998;32(2):230–7.
15. Maloney DG, Grillo-Lopez AJ, White CA, Bodkin D, Schilder
RJ, Neidhart JA, et al. IDEC-C2B8 (Rituximab) anti-CD20 monoclonal antibody therapy in patients with relapsed low-grade nonHodgkin’s lymphoma. Blood. 1997;90(6):2188–95.
16. Bagga A, Sinha A, Moudgil A. Rituximab in patients with
the steroid-resistant nephrotic syndrome. N Engl J Med.
2007;356(26):2751–2. https://doi.org/10.1056/NEJMc063706.
17. Gulati A, Sinha A, Jordan SC, Hari P, Dinda AK, Sharma S,
et al. Efficacy and safety of treatment with rituximab for difficult
steroid-resistant and -dependent nephrotic syndrome: multicentric
report. Clin J Am Soc Nephrol. 2010;5(12):2207–12. https://doi.
org/10.2215/CJN.03470410.
18. Ito S, Kamei K, Ogura M, Udagawa T, Fujinaga S, Saito M, et al.
Survey of rituximab treatment for childhood-onset refractory
nephrotic syndrome. Pediatr Nephrol. 2013;28(2):257–64. https://
doi.org/10.1007/s00467-012-2319-1.
19. Kamei K, Okada M, Sato M, Fujimaru T, Ogura M, Nakayama
M, et al. Rituximab treatment combined with methylprednisolone
pulse therapy and immunosuppressants for childhood steroidresistant nephrotic syndrome. Pediatr Nephrol. 2014;29(7):1181–
7. https://doi.org/10.1007/s00467-014-2765-z.
20. Magnasco A, Ravani P, Edefonti A, Murer L, Ghio L, Belingheri
M, et al. Rituximab in children with resistant idiopathic nephrotic
syndrome. J Am Soc Nephrol. 2012;23(6):1117–24. https://doi.
org/10.1681/ASN.2011080775.
21. Nakayama M, Kamei K, Nozu K, Matsuoka K, Nakagawa A, Sako
M, et al. Rituximab for refractory focal segmental glomerulosclerosis. Pediatr Nephrol. 2008;23(3):481–5. https://d oi.o rg/1 0.1 007/
s00467-007-0640-x.
22. Trautmann A, Vivarelli M, Samuel S, Gipson D, Sinha A, Schaefer
F, et al. IPNA clinical practice recommendations for the diagnosis
and management of children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2020;35(8):1529–61. https://doi.org/10.
1007/s00467-020-04519-1.
23. Fujinaga S, Sakuraya K. Repeated administrations of rituximab along with steroids and immunosuppressive agents in
refractory steroid-resistant nephrotic syndrome. Indian Pediatr.
2017;54(1):49–50. https://doi.org/10.1007/s13312-017-0996-3.
24. Nagano C, Yamamura T, Horinouchi T, Aoto Y, Ishiko S, Sakakibara N, et al. Comprehensive genetic diagnosis of Japanese
patients with severe proteinuria. Sci Rep. 2020;10(1):270. https://
doi.org/10.1038/s41598-019-57149-5.
25. Abe T, Matsuo H, Abe R, Abe S, Asada H, Ashida A, et al. The
Japanese Society for Apheresis clinical practice guideline for therapeutic apheresis. Ther Apher Dial. 2021;25(6):728–876. https://
doi.org/10.1111/1744-9987.13749.
26. Iijima K, Sako M, Nozu K, Mori R, Tuchida N, Kamei K, et al.
Rituximab for childhood-onset, complicated, frequently relapsing
nephrotic syndrome or steroid-dependent nephrotic syndrome: a
multicentre, double-blind, randomised, placebo-controlled trial.
Lancet. 2014;384(9950):1273–81. https://d oi.o rg/1 0.1 016/S
0140-
6736(14)60541-9.
27. Suri M, Tran K, Sharma AP, Filler G, Grimmer J. Remission
of steroid-resistant nephrotic syndrome due to focal and segmental glomerulosclerosis using rituximab. Int Urol Nephrol.
2008;40(3):807–10. https://doi.org/10.1007/s11255-008-9393-0.
28. Kamei K, Ishikura K, Sako M, Ito S, Nozu K, Iijima K. Rituximab therapy for refractory steroid-resistant nephrotic syndrome
in children. Pediatr Nephrol. 2020;35(1):17–24. https://doi.org/
10.1007/s00467-018-4166-1.
29. Kobayashi M, Kageyama Y, Ando T, Sakamoto J, Kimura S.
All-case Japanese post-marketing surveillance of the real-world
safety and efficacy of rituximab treatment in patients with refractory nephrotic syndrome. Clin Exp Nephrol. 2021;25(8):854–64.
https://doi.org/10.1007/s10157-021-02035-6.
30. Ohyama R, Fujinaga S, Sakuraya K, Hirano D, Ito S. Predictive
factors of long-term disease remission after rituximab administration in patients with childhood-onset complicated steroid-dependent nephrotic syndrome: a single-center retrospective study. Clin
Exp Nephrol. 2023;27(10):865–72. https://d oi.o rg/1 0.1 007/
s10157-023-02374-6.
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jurisdictional claims in published maps and institutional affiliations.
Authors and Affiliations
Kandai Nozu1 · Mayumi Sako2 · Seiji Tanaka3 · Yuji Kano4 · Yoko Ohwada4 · Tamaki Morohashi5 · Riku Hamada6 ·
Yasufumi Ohtsuka7 · Masafumi Oka7 · Koichi Kamei8 · Aya Inaba9 · Shuichi Ito9 · Tomoyuki Sakai10 · Hiroshi Kaito11 ·
Yuko Shima12 · Kenji Ishikura13 · Hidefumi Nakamura14 · Koichi Nakanishi15 · Tomoko Horinouchi1 ·
Akihide Konishi16 · Takashi Omori16 · Kazumoto Iijima1
* Kandai Nozu
nozu@med.kobe-u.ac.jp
Department of Pediatrics, Kobe University Graduate
School of Medicine, 7‑5‑1 Kusunoki‑Cho, Chuo‑Ku,
Kobe 650‑0017, Japan
Division for Clinical Trials, Department of Clinical Research
Promotion, Clinical Research Center, National Center
for Child Health and Development, Tokyo, Japan
Department of Pediatrics, Kurume University School
of Medicine, Kurume, Japan
13
Clinical and Experimental Nephrology
Department of Pediatrics, Dokkyo Medical University School
of Medicine, Tochigi, Japan
11
Department of Pediatrics, Nihon University School
of Medicine, Tokyo, Japan
12
Department of Nephrology, Tokyo Metropolitan Children’s
Medical Center, Tokyo, Japan
13
Department of Pediatrics, Saga University School
of Medicine, Saga, Japan
14
Division of Nephrology and Rheumatology, National Center
for Child Health and Development, Tokyo, Japan
15
Department of Pediatrics, Graduate School of Medicine,
Yokohama City University, Yokohama, Japan
10
Department of Pediatrics, Shiga University of Medical
Science, Shiga, Japan
13
Department of Nephrology, Hyogo Prefectural Kobe
Children’s Hospital, Kobe, Japan
Department of Pediatrics, Wakayama Medical University,
Wakayama, Japan
Department of Pediatrics, Kitasato University School
of Medicine, Kanagawa, Japan
Clinical Research Center, National Center for Child Health
and Development, Tokyo, Japan
Department of Child Health and Welfare (Pediatrics),
Graduate School of Medicine, University of the Ryukyus,
Okinawa, Japan
16
Clinical and Translational Research Center, Kobe University
Hospital, Kobe, Japan
...