{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T23:02:00Z","timestamp":1793314920668,"version":"4.5.0"},"reference-count":22,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2025,3,25]],"date-time":"2025-03-25T00:00:00Z","timestamp":1742860800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,3,25]],"date-time":"2025-03-25T00:00:00Z","timestamp":1742860800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","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":[[2025,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Background<\/jats:title>\n            <jats:p>This study is a continuation of the Japan Rapidly Progressive GlomeruloNephritis (RPGN) Working Group\u2019s chronological nationwide survey.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods<\/jats:title>\n            <jats:p>We analyzed 1,660 RPGN cases from 2016\u20132019 and compared them to 4,179 cases from five earlier periods (1989\u20131998, 1999\u20132001, 2002\u20132008, 2009\u20132011, 2012\u20132015). Data on causative diseases, clinical severity, 24-month life and renal survival, and treatment details were collected and compared.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results<\/jats:title>\n            <jats:p>The most recent cohort showed an older median age at onset (median age 74\u00a0years), with improved serum creatinine levels (median 2.5\u00a0mg\/dL). Cumulative survival at 24\u00a0months remained stable (periods 1989\u20131998, 1999\u20132001, 2002\u20132008, 2009\u20132011, 2012\u20132015, 2016\u20132019 were each 72.0%, 72.9%, 77.7%, 83.0%, 84.9%, 83.5%, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01), while renal survival showed a favorable trend in the most recent periods (there were each 68.7%, 75.4%, 76.7%, 73.4%, 78.2%, 78.4%, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01). Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV)-RPGN had similar outcomes to the overall cohort. Increased rituximab use was observed, with no significant differences in life and renal prognosis between rituximab (RIX) and cyclophosphamide (CY). In severe renal impairment (Cre\u2009\u2265\u20096), renal prognosis was better in the CY or RIX use group than in the non-use group (<jats:italic>p<\/jats:italic>\u2009=\u20090.035, 0.025). Anti-glomerular basement membrane disease had a poorer renal prognosis compared to other causes.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p>Despite an increasingly older age of onset, both life and renal prognoses for new-onset AAV-RPGN from 2016 to 2019 remain comparable to the best in previous surveys, due to the impact of constant improvements in early diagnosis and changes in treatment.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s10157-025-02643-6","type":"journal-article","created":{"date-parts":[[2025,3,28]],"date-time":"2025-03-28T02:33:57Z","timestamp":1743129237000},"page":"937-952","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Temporal changes of the life and renal prognoses of patients with rapidly progressive glomerulonephritis in Japan, 1989\u20132019"],"prefix":"10.1007","volume":"29","author":[{"given":"Kentaro","family":"Nakajima","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuzo","family":"Kaneko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6709-4000","authenticated-orcid":false,"given":"Joichi","family":"Usui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naotake","family":"Tsuboi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hitoshi","family":"Sugiyama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shoichi","family":"Maruyama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshitaka","family":"Isaka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ichiei","family":"Narita","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kunihiro","family":"Yamagata","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,3,25]]},"reference":[{"key":"2643_CR1","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, Narita I. Epidemiology and temporal changes in the prognosis of rapidly progressive glomerulonephritis in Japan: a Nationwide 1989\u20132015 Survey. Clin Exp Nephrol. 2022;26:234\u201346.","journal-title":"Clin Exp Nephrol"},{"key":"2643_CR2","doi-asserted-by":"crossref","unstructured":"Koyama A, Yamagata K, Makino H, Arimura Y, Wada T, Nitta K, Nihei H, Muso E, Taguma Y, Shigematsu H, Sakai H, Tomino Y, Matsuo S, Japan RPGN Registry Group. A nationwide survey of rapidly progressive glomerulonephritis in Japan: Etiology, prognosis and treatment diversity. Clin Exp Nephrol 2009;13:633\u201350.","DOI":"10.1007\/s10157-009-0201-7"},{"key":"2643_CR3","doi-asserted-by":"publisher","first-page":"580","DOI":"10.1007\/s10157-012-0598-2","volume":"16","author":"K Yamagata","year":"2012","unstructured":"Yamagata K, Usui J, Saito C, Yamaguchi N, Hirayama K, Mase K, Kobayashi M, Koyama A, Sugiyama H, Nitta K, Wada T, Muso E, Arimura Y, Makino H, Matsuo S. 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Informed consent was obtained from all individual patients included in this study; each participating facility disclosed the purpose of the survey, method, survey period, survey contents, and privacy protection, and they provided all registered patients an opportunity to opt out.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}}]}}