Skip to main content

Japanese clinical practice patterns of primary nephrotic syndrome 2021: a web-based questionnaire survey of certified nephrologists



With the publication of the “Evidence-Based Clinical Practice Guideline for Nephrotic Syndrome 2020,” we examined nephrologists’ adherence to the recommendations of four of its clinical questions (CQs).


This was a cross-sectional web-based survey conducted between November and December 2021. The target population comprised nephrologists certified by the Japanese Society of Nephrology who were recruited using convenience sampling. The participants answered six items regarding the four CQs about adult patients with nephrotic syndrome and their characteristics.


In total, 434 respondents worked in at least 306 facilities, of whom 386 (88.9%) provided outpatient care for primary nephrotic syndrome. Of these patients, 179 (41.2%) answered that they would not measure anti- phospholipase A2 receptor antibody levels in cases of suspected primary membranous nephropathy (MN) in which kidney biopsy was not possible (CQ1). Regarding immunosuppressants as maintenance therapy after relapse of minimal change nephrotic syndrome (CQ2), cyclosporine was the most common choice (290 [72.5%] and 300 [75.0%] of 400 respondents after the first and second relapses, respectively). The most common treatment for steroid-resistant cases of primary focal segmental glomerulosclerosis (CQ3) was cyclosporine (323 of 387, 83.5%). For the initial treatment of primary MN with nephrotic-range proteinuria (CQ4), corticosteroid monotherapy was the most common choice (240 of 403, 59.6%), followed by corticosteroid and cyclosporine (114, 28.3%).


Gaps in recommendations and practices regarding serodiagnosis and treatment of MN (i.e., CQ1 and 4) are observed, suggesting the need to address the barriers to their insurance reimbursement and the lack of evidence behind them.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2

Data availability

The data underlying this article will be shared upon reasonable request from the corresponding author.


  1. Sakai H, Kurokawa K, Saito T, Shiiki H, Nishi S, Mitarai T, et al. Guidelines for the management of refractory nephrotic syndrome–from investigations between 1997 and 2001–research project team for progressive renal lesions in the Ministry of Health, Labor and Welfare, Japan. Nihon Jinzo Gakkai Shi. 2002;44(8):751–61.

    PubMed  Google Scholar 

  2. Nishi S, Ubara Y, Utsunomiya Y, Okada H, Obata Y, Kai H, et al. Evidence-based clinical practice guidelines for nephrotic syndrome 2014. Clin Exp Nephrol. 2016;20(3):342–70.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Maruyama S. Committee of Clinical Practice Guideline for Nephrotic Syndrome 2017, editors. Evedence-based clinical practice guideline for nephrotic syndrome 2017. Tokyo: Tokyo Igakusha. 2017.

  4. Wada T, Ishimoto T, Nakaya I, Kawaguchi T, Sofue T, Shimizu S, et al. A digest of the evidence-based clinical practice guideline for nephrotic syndrome 2020. Clin Exp Nephrol. 2021;25(12):1277–85.

    Article  PubMed  Google Scholar 

  5. Narita I. Committee of Clinical Practice Guideline for Nephrotic Syndrome 2020, editors. Evedence-based clinical practice guideline for nephrotic syndrome 2017. Tokyo: Tokyo Igakusha. 2020.

  6. Lamer A, Laurent G, Pelayo S, El Amrani M, Chazard E, Marcilly R. Exploring patient path through Sankey diagram: a proof of concept. Stud Health Technol Inform. 2020;270:218–22.

    Article  PubMed  Google Scholar 

  7. Du Y, Li J, He F, Lv Y, Liu W, Wu P, et al. The diagnosis accuracy of PLA2R-AB in the diagnosis of idiopathic membranous nephropathy: a meta-analysis. PLoS One. 2014;9(8):e104936.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Kidney Disease: Improving Global Outcomes Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021;100(4S):S1-276.

    Article  Google Scholar 

  9. Eguchi A, Takei T, Yoshida T, Tsuchiya K, Nitta K. Combined cyclosporine and prednisolone therapy in adult patients with the first relapse of minimal-change nephrotic syndrome. Nephrol Dial Transplant. 2010;25(1):124–9.

    Article  CAS  PubMed  Google Scholar 

  10. Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE, et al. A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America nephrotic syndrome study group. Kidney Int. 1999;56(6):2220–6.

    Article  CAS  PubMed  Google Scholar 

  11. Gipson DS, Trachtman H, Kaskel FJ, Greene TH, Radeva MK, Gassman JJ, et al. Clinical trial of focal segmental glomerulosclerosis in children and young adults. Kidney Int. 2011;80(8):868–78.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Yamaguchi M, Ando M, Yamamoto R, Akiyama S, Kato S, Katsuno T, et al. Patient age and the prognosis of idiopathic membranous nephropathy. PLoS One. 2014;9(10):e110376.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Bech AP, Hofstra JM, Brenchley PE, Wetzels JF. Association of anti-PLA(2)R antibodies with outcomes after immunosuppressive therapy in idiopathic membranous nephropathy. Clin J Am Soc Nephrol. 2014;9(8):1386–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. De Vriese AS, Glassock RJ, Nath KA, Sethi S, Fervenza FC. A proposal for a serology-based approach to membranous nephropathy. J Am Soc Nephrol. 2017;28(2):421–30.

    Article  PubMed  Google Scholar 

  15. Akiyama S, Akiyama M, Imai E, Ozaki T, Matsuo S, Maruyama S. Prevalence of anti-phospholipase A2 receptor antibodies in Japanese patients with membranous nephropathy. Clin Exp Nephrol. 2015;19(4):653–60.

    Article  CAS  PubMed  Google Scholar 

  16. Saito T, Iwano M, Matsumoto K, Mitarai T, Yokoyama H, Yorioka N, et al. Significance of combined cyclosporine−prednisolone therapy and cyclosporine blood concentration monitoring for idiopathic membranous nephropathy with steroid-resistant nephrotic syndrome: a randomized controlled multicenter trial. Clin Exp Nephrol. 2014;18(5):784–94.

    Article  CAS  PubMed  Google Scholar 

  17. Xu J, Hu X, Xie J, Chen N. Management of membranous nephropathy in Asia. Kidney Dis (Basel). 2015;1(2):119–25.

    Article  PubMed  Google Scholar 

  18. Lin S, Li H-Y, Zhou T, Lin W. Efficacy and safety of cyclosporine A in the treatment of idiopathic membranous nephropathy in an Asian population. Drug Des Devel Ther. 2019;13:2305–30.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Fervenza FC, Appel GB, Barbour SJ, Rovin BH, Lafayette RA, Aslam N, et al. Rituximab or cyclosporine in the treatment of membranous nephropathy. N Engl J Med. 2019;381(1):36–46.

    Article  CAS  PubMed  Google Scholar 

  20. 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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Eba J, Nakamura K. Overview of the ethical guidelines for medical and biological research involving human subjects in Japan. Jpn J Clin Oncol. 2022;52(6):539–44.

    Article  PubMed  PubMed Central  Google Scholar 

Download references


This study was supported in part by a Grant-in-Aid for Intractable Renal Diseases Research, Research on Rare and Intractable Diseases, and Health and Labor Sciences Research Grants from the Ministry of Health, Labour and Welfare of Japan (ID 20FC1045). This funder had no role in the design, conduct, or reporting of this study. We would like to thank Editage ( for the English language editing.

Author information

Authors and Affiliations



Study concept and design: TW, SS, TI, and NK. Data collection: TW, SS, and NK. Data analysis: SS, SS, and NK. Data interpretation and manuscript preparation: TW, SS, MK, TS, HN, SS, IN, YO, TI, KF, HO, and NK.

Corresponding author

Correspondence to Takehiko Wada.

Ethics declarations

Conflict of interest

Honoraria: Hirokazu Okada (AstraZeneca, Daiichi Sankyo, Kyowa Kirin, Mitsubishi Tanabe Pharm, Torii Pharmaceutical), Noriaki Kurita (GlaxoSmithKline), Research funding: Kengo Furuichi (Chugai Pharmaceutical, Daiichi Sankyo), Hirokazu Okada (Kyowa Kirin), Subsidies or donations: Hirokazu Okada (Bayer, Chugai Pharmaceutical, Kyowa Kirin, Ono Pharmaceutical).

Ethical approval

This study was an anonymous survey of healthcare professionals to describe the practice of primary nephrotic syndrome and was considered outside the scope of the ethical review according to the Ethical Guidelines for Medical and Biological Research Involving Human Subjects [21]. The possibility of academic publication of the survey results was described at the beginning of the questionnaire, and only those who provided consent to complete the survey were included.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file1 (PDF 210 KB)

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wada, T., Shimizu, S., Koizumi, M. et al. Japanese clinical practice patterns of primary nephrotic syndrome 2021: a web-based questionnaire survey of certified nephrologists. Clin Exp Nephrol 27, 767–775 (2023).

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: