{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T22:47:15Z","timestamp":1793141235872,"version":"4.5.0"},"reference-count":45,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,4,2]],"date-time":"2026-04-02T00:00:00Z","timestamp":1775088000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>The long-term prognosis of patients with anti-neutrophil cytoplasmic antibody\u2013associated vasculitis (AAV) has historically been poor. In recent years, the widespread use of novel targeted therapies has led to an increased emphasis on reduced-dose glucocorticoid (GC) strategies; however, owing to regional differences in the frequency and clinical characteristics of AAV subtypes, contemporary real-world evidence on long-term outcomes in Japanese patient populations remains limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>The primary outcome was to elucidate subtype-specific clinical characteristics and five-year overall and relapse-free survival in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Secondary outcomes included temporal changes in treatment practices and prognosis, stratified by year of diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a multicentre retrospective study using data from the REVEAL cohort. A total of 460 newly diagnosed, treatment-na\u00efve cases were included (microscopic polyangiitis (MPA), n = 283; granulomatosis with polyangiitis (GPA), n = 66; eosinophilic granulomatosis with polyangiitis (EGPA), n = 111). Clinical characteristics, five-year relapse-free and overall survival were evaluated. To assess temporal changes in treatment practices and prognosis, patients were stratified by median year of diagnosis (2018) into pre-2018 and post-2019 groups.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      MPA showed the poorest prognosis among AAV subtypes, with a five-year overall survival rate of 67.6%. Older age and impaired renal function were independently associated with increased mortality (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0&amp;lt;\u00a00.001 and\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.0079, respectively). Over time, GC exposure was significantly reduced in the post-2019 group (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.012), accompanied by fewer infection-related hospitalisations (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0&amp;lt;\u00a00.001). However, five-year overall and relapse-free survival did not differ between the pre-2018 and post-2019 groups. Notably, BVAS at 6 and 12 months were significantly higher (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.0070 and\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.0057, respectively), and vasculitis-related mortality was more frequent in the post-2019 group (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.0057).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>MPA remained the AAV subtype with the poorest prognosis. Although GC exposure has decreased in recent years, this trend was not associated with improved clinical outcomes in the present cohort.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1807423","type":"journal-article","created":{"date-parts":[[2026,4,2]],"date-time":"2026-04-02T05:24:00Z","timestamp":1775107440000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Long-term outcomes remain unchanged despite reduced glucocorticoid exposure in ANCA-associated vasculitis: the multicentre REVEAL cohort study"],"prefix":"10.3389","volume":"17","author":[{"given":"Mayu","family":"Shiomi","sequence":"first","affiliation":[{"name":"Department of Clinical Immunology, Osaka Metropolitan University Graduate School of Medicine","place":["Osaka, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryu","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of 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