{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T10:12:02Z","timestamp":1774260722528,"version":"3.50.1"},"reference-count":14,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2026,2,15]],"date-time":"2026-02-15T00:00:00Z","timestamp":1771113600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,2,15]],"date-time":"2026-02-15T00:00:00Z","timestamp":1771113600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100020050","name":"Japan Intractable Diseases Research Foundation","doi-asserted-by":"publisher","award":["23FC1048"],"award-info":[{"award-number":["23FC1048"]}],"id":[{"id":"10.13039\/100020050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Exp Nephrol"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Primary membranoproliferative glomerulonephritis (MPGN) is a rare progressive kidney disease that often leads to end-stage kidney disease. Our previous nationwide registry study (Report 1, 2015\u20132018) characterized initial demographics and treatment patterns. In this study (Report 2), we aimed to update these findings, focusing on newly registered cases (2017\u20132021).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Personal clinical records of patients with primary MPGN between 2017 and 2021 were obtained from the national registry organized by the Japanese Ministry of Health, Labour and Welfare. Characteristics of primary MPGN throughout Japan were investigated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 210 patients (median age, 49\u00a0years; male, 51%) with newly registered primary MPGN were identified. Nephrotic syndrome was present in 83.8% of patients at enrollment. Initial treatment frequently included corticosteroids (63.8%), with a modest increase in intravenous methylprednisolone pulse therapy (41.4%) compared with that in Report 1. The incidence of hemodialysis was 7.1%. Compared with those in Report 1, the demographic patterns were similar; however, nephrotic presentations were more common. Cyclosporine and mizoribine usage were significantly higher in the pediatric group (&lt;\u200918\u00a0years,\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200944) compared to the corresponding usage in the older adult group (\u2265\u200965\u00a0years,\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200975). The mean dosage of oral prednisolone and other immunosuppressants during initial treatment did not differ among the four age groups.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Compared with the earlier report (Report 1), patients with newly registered primary MPGN presented with nephrotic syndrome more often, highlighting the continued risk of poor prognosis and the need for more refined therapeutic approaches.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s10157-026-02828-7","type":"journal-article","created":{"date-parts":[[2026,2,15]],"date-time":"2026-02-15T16:10:18Z","timestamp":1771171818000},"page":"590-596","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Initial treatment patterns of primary membranoproliferative glomerulonephritis in Japan (2017\u20132021): an updated analysis based on nationwide personal clinical records"],"prefix":"10.1007","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5398-3667","authenticated-orcid":false,"given":"Naoki","family":"Nakagawa","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keiju","family":"Hiromura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshitaka","family":"Isaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,2,15]]},"reference":[{"key":"2828_CR1","unstructured":"Kidney Disease: Improving Global Outcomes Glomerular Diseases Work G. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021;100:S1-S276."},{"key":"2828_CR2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0257397","volume":"16","author":"N Nakagawa","year":"2021","unstructured":"Nakagawa N, Mizuno M, Kato S, Maruyama S, Sato H, Nakaya I, et al. Demographic, clinical characteristics and treatment outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulonephritis in Japan: a retrospective analysis of data from the Japan Renal Biopsy Registry. PLoS ONE. 2021;16:e0257397.","journal-title":"PLoS ONE"},{"key":"2828_CR3","doi-asserted-by":"publisher","first-page":"797","DOI":"10.1007\/s10157-017-1513-7","volume":"22","author":"N Nakagawa","year":"2018","unstructured":"Nakagawa N, Hasebe N, Hattori M, Nagata M, Yokoyama H, Sato H, et al. Clinical features and pathogenesis of membranoproliferative glomerulonephritis: a nationwide analysis of the Japan renal biopsy registry from 2007 to 2015. Clin Exp Nephrol. 2018;22:797\u2013807.","journal-title":"Clin Exp Nephrol"},{"key":"2828_CR4","doi-asserted-by":"publisher","first-page":"1119","DOI":"10.1056\/NEJMra1108178","volume":"366","author":"S Sethi","year":"2012","unstructured":"Sethi S, Fervenza FC. Membranoproliferative glomerulonephritis--a new look at an old entity. N Engl J Med. 2012;366:1119\u201331.","journal-title":"N Engl J Med"},{"key":"2828_CR5","doi-asserted-by":"publisher","first-page":"928","DOI":"10.1007\/s10157-023-02387-1","volume":"27","author":"N Nakagawa","year":"2023","unstructured":"Nakagawa N, Kimura T, Sakate R, Isaka Y, Narita I. Demographics and treatment of patients with primary membranoproliferative glomerulonephritis in Japan using a national registry of clinical personal records. Clin Exp Nephrol. 2023;27:928\u201335.","journal-title":"Clin Exp Nephrol"},{"key":"2828_CR6","doi-asserted-by":"crossref","unstructured":"Vivarelli M, Barratt J, Beck LH, Jr., Fakhouri F, Gale DP, Goicoechea de Jorge E, et al. The role of complement in kidney disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2024;106:369\u201391.","DOI":"10.1016\/j.kint.2024.05.015"},{"key":"2828_CR7","doi-asserted-by":"publisher","first-page":"1135","DOI":"10.1016\/j.kint.2020.05.053","volume":"98","author":"F Fakhouri","year":"2020","unstructured":"Fakhouri F, Le Quintrec M, Fremeaux-Bacchi V. Practical management of C3 glomerulopathy and Ig-mediated MPGN: facts and uncertainties. Kidney Int. 2020;98:1135\u201348.","journal-title":"Kidney Int"},{"key":"2828_CR8","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.1016\/S0140-6736(25)01148-1","volume":"406","author":"D Kavanagh","year":"2025","unstructured":"Kavanagh D, Bomback AS, Vivarelli M, Nester CM, Remuzzi G, Zhao MH, et al. Oral iptacopan therapy in patients with C3 glomerulopathy: a randomised, double-blind, parallel group, multicentre, placebo-controlled, phase 3 study. Lancet. 2025;406:1587\u201398.","journal-title":"Lancet"},{"key":"2828_CR9","doi-asserted-by":"publisher","first-page":"519","DOI":"10.1007\/s10157-023-02337-x","volume":"27","author":"T Kimura","year":"2023","unstructured":"Kimura T, Ikeuchi H, Yoshino M, Sakate R, Maruyama S, Narita I, et al. Profiling of kidney involvement in systemic lupus erythematosus by deep learning using the National Database of Designated Incurable Diseases of Japan. Clin Exp Nephrol. 2023;27:519\u201327.","journal-title":"Clin Exp Nephrol"},{"key":"2828_CR10","doi-asserted-by":"publisher","first-page":"1","DOI":"10.2176\/nmc.st.2016-0135","volume":"57","author":"Y Kanatani","year":"2017","unstructured":"Kanatani Y, Tomita N, Sato Y, Eto A, Omoe H, Mizushima H. National Registry of Designated Intractable Diseases in Japan: present status and future prospects. Neurol Med Chir (Tokyo). 2017;57:1\u20137.","journal-title":"Neurol Med Chir (Tokyo)"},{"key":"2828_CR11","doi-asserted-by":"publisher","first-page":"1277","DOI":"10.1007\/s10157-021-02098-5","volume":"25","author":"T Wada","year":"2021","unstructured":"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;25:1277\u201385.","journal-title":"Clin Exp Nephrol"},{"key":"2828_CR12","first-page":"671","volume":"26","author":"H Sakaguchi","year":"1984","unstructured":"Sakaguchi H, Hazikano H, Hasegawa O, Ito H. Histological subtype of mesangiocapillary glomerulonephritis (MPGN type 1). Nihon Jinzo Gakkai Shi. 1984;26:671\u201389.","journal-title":"Nihon Jinzo Gakkai Shi"},{"key":"2828_CR13","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1007\/s10157-015-1216-x","volume":"20","author":"S Nishi","year":"2016","unstructured":"Nishi S, Ubara Y, Utsunomiya Y, Okada K, Obata Y, Kai H, et al. Evidence-based clinical practice guidelines for nephrotic syndrome 2014. Clin Exp Nephrol. 2016;20:342\u201370.","journal-title":"Clin Exp Nephrol"},{"key":"2828_CR14","doi-asserted-by":"publisher","first-page":"283","DOI":"10.5582\/irdr.2021.01145","volume":"10","author":"T Sawakami","year":"2021","unstructured":"Sawakami T. Current status of specific pediatric chronic diseases in Japan: national measures, disease types, treatment availability, copayment assistance, and research. Intractable Rare Dis Res. 2021;10:283\u20137.","journal-title":"Intractable Rare Dis Res"}],"container-title":["Clinical and Experimental Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10157-026-02828-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10157-026-02828-7","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10157-026-02828-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T09:28:30Z","timestamp":1774258110000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10157-026-02828-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,15]]},"references-count":14,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4]]}},"alternative-id":["2828"],"URL":"https:\/\/doi.org\/10.1007\/s10157-026-02828-7","relation":{},"ISSN":["1342-1751","1437-7799"],"issn-type":[{"value":"1342-1751","type":"print"},{"value":"1437-7799","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,15]]},"assertion":[{"value":"22 October 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"9 February 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 February 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All the authors have declared no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"This study was conducted in accordance with the ethical principles embodied in the Declaration of Helsinki. The study protocol was approved by the Ethics Review Board of the Japanese Society of Nephrology (approval number: 124 (70-7); approval date: December 23, 2024). The requirement for informed consent was waived because all data included in this dataset were de-identified.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical statement"}}]}}