{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,19]],"date-time":"2026-02-19T06:54:24Z","timestamp":1771484064021,"version":"3.50.1"},"reference-count":13,"publisher":"Springer Science and Business Media LLC","issue":"8","license":[{"start":{"date-parts":[[2025,3,27]],"date-time":"2025-03-27T00:00:00Z","timestamp":1743033600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,3,27]],"date-time":"2025-03-27T00:00:00Z","timestamp":1743033600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"a Grant-in-Aid for Intractable Renal Diseases Research, Research on Rare and Intractable Diseases, Health and Labour Sciences Research Grants from the Ministry of Health, Labour and Welfare of Japan"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Exp Nephrol"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Background<\/jats:title>\n            <jats:p>As a joint project with the Ministry of Health, Labour and Welfare (MHLW), the Research Group on Intractable Renal Diseases is examining the feasibility of utilizing its personal clinical records database. We examine the validity of the initial-treatment data from the personal clinical records of patients with new-onset rapidly progressive glomerulonephritis (RPGN).<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods<\/jats:title>\n            <jats:p>Personal clinical records for patients with either RPGN or anti-glomerular basement membrane (GBM) antibody nephritis were used. The data from 454 newly enrolled RPGN patients were compiled for analysis in 2 cohort studies (CS1 for all case analysis and CS2 for selective analysis of new-onset cases).<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results<\/jats:title>\n            <jats:p>In CS1, the serotypes of the 362 registered RPGN cases included 200 myeloperoxidase (MPO)-ANCA-positive, 98 anti-GBM-positive, and 9 proteinase-3 (PR3)-ANCA-positive cases, etc. CS2 included 96 of the MPO-ANCA-positive RPGN and 55 of the anti-GBM antibody-positive RPGN cases. For the initial treatment of MPO-ANCA-positive RPGN, the rates of glucocorticoid (GC) and GC pulse treatment were similar between the personal clinical records database and the nationwide questionnaire survey, but the rates of intravenous cyclophosphamide (CY) or rituximab were statistically significant lower in the personal clinical records database. For the initial treatment of anti-GBM antibody-positive RPGN, the rate of plasma exchange was similar between the two databases, but the rates of GC and per os CY tended to be lower in the personal clinical records database, although not statistically significant.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion<\/jats:title>\n            <jats:p>Clear differences in initial treatment for new-onset RPGN patients were found between a personal clinical records database and another reported database.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s10157-025-02657-0","type":"journal-article","created":{"date-parts":[[2025,3,30]],"date-time":"2025-03-30T06:08:14Z","timestamp":1743314894000},"page":"1095-1101","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Status of initial treatment for rapidly progressive glomerulonephritis in Japan: analysis of a personal clinical records database"],"prefix":"10.1007","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6709-4000","authenticated-orcid":false,"given":"Joichi","family":"Usui","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomonori","family":"Kimura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kunihiro","family":"Yamagata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kentaro","family":"Nakajima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shuzo","family":"Kaneko","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ken-ei","family":"Sada","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naotake","family":"Tsuboi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirokazu","family":"Okada","sequence":"additional","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"}]},{"given":"Ichiei","family":"Narita","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,3,27]]},"reference":[{"issue":"6","key":"2657_CR1","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1007\/s10157-009-0201-7","volume":"13","author":"A Koyama","year":"2009","unstructured":"Koyama A, Yamagata K, Makino H, Arimura Y, Wada T, Nitta K, et al. A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity. Clin Exp Nephrol. 2009;13(6):633\u201350.","journal-title":"Clin Exp Nephrol"},{"issue":"3","key":"2657_CR2","doi-asserted-by":"publisher","first-page":"234","DOI":"10.1007\/s10157-021-02148-y","volume":"26","author":"S Kaneko","year":"2022","unstructured":"Kaneko S, Yamagata K, Usui J, Tsuboi N, Sugiyama H, Maruyama S, et al. Epidemiology and temporal changes in the prognosis of rapidly progressive glomerulonephritis in Japan: a nationwide 1989\u20132015 survey. Clin Exp Nephrol. 2022;26(3):234\u201346.","journal-title":"Clin Exp Nephrol"},{"issue":"12","key":"2657_CR3","doi-asserted-by":"publisher","first-page":"1286","DOI":"10.1007\/s10157-021-02096-7","volume":"25","author":"J Usui","year":"2021","unstructured":"Usui J, Kawashima S, Sada KE, Miyawaki Y, Nakazawa D, Itabashi M, et al. A digest of the evidence-based clinical practice guideline for rapidly progressive glomerulonephritis 2020. Clin Exp Nephrol. 2021;25(12):1286\u201391.","journal-title":"Clin Exp Nephrol"},{"issue":"8","key":"2657_CR4","doi-asserted-by":"publisher","first-page":"1366","DOI":"10.1002\/art.41773","volume":"73","author":"SA Chung","year":"2021","unstructured":"Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, et al. 2021 American college of rheumatology\/vasculitis foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021;73(8):1366\u201383.","journal-title":"Arthritis Rheumatol"},{"key":"2657_CR5","doi-asserted-by":"publisher","DOI":"10.1136\/rmdopen-2023-003082","author":"JH Schirmer","year":"2023","unstructured":"Schirmer JH, Sanchez-Alamo B, Hellmich B, Jayne D, Monti S, Luqmani RA, et al. Systematic literature review informing the 2022 update of the EULAR recommendations for the management of ANCA-associated vasculitis (AAV): part 1-treatment of granulomatosis with polyangiitis and microscopic polyangiitis. RMD Open. 2023. https:\/\/doi.org\/10.1136\/rmdopen-2023-003082.","journal-title":"RMD Open"},{"issue":"5","key":"2657_CR6","doi-asserted-by":"publisher","first-page":"646","DOI":"10.1007\/s10157-012-0740-1","volume":"17","author":"K Yamagata","year":"2013","unstructured":"Yamagata K, Usui J, Sugiyama H, Nitta K, Wada T, Muso E, et al. Clinical findings on ANCA-associated renal vasculitis from the Japan RPGN registry obtained via a questionnaire survey. 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