{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T20:58:09Z","timestamp":1790456289400,"version":"4.1.0"},"reference-count":40,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T00:00:00Z","timestamp":1779148800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T00:00:00Z","timestamp":1779148800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Japan Pancreas Society"},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["23FC1015"],"award-info":[{"award-number":["23FC1015"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Gastroenterol"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>To update the clinic-epidemiological features of autoimmune pancreatitis (AIP) in Japan, we conducted the fifth nationwide survey.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We performed a two-stage survey of patients with AIP who were treated at selected hospitals in 2021. The first-stage survey estimated the total number of patients with AIP, and the second-stage survey analyzed detailed clinical characteristics.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The estimated number of patients with AIP in 2021 was 16,750 (prevalence, 13.3 per 100,000 persons), representing a 25% increase from 2016. The number of newly diagnosed cases was 4210 (annual incidence, 3.4 per 100,000 persons). Clinical data were available for 2833 patients. The male-to-female ratio was 3.32:1, and the mean age in 2021 and the mean age at AIP diagnosis were 71.2 and 66.3\u00a0years, respectively. At diagnosis, 58.6% of patients were symptomatic, approximately half of whom presented with jaundice. Other organ involvement was observed in 67.8% of patients, most commonly sclerosing cholangitis. Histopathological examination was performed in 84.1% of cases, predominantly using endoscopic ultrasonography-guided tissue acquisition. Initial glucocorticoid therapy was administered to 84.2% of patients, and 93.8% received maintenance therapy. Kaplan\u2013Meier analysis demonstrated a cumulative relapse incidence of 12.5%, 20.7%, 36.0%, 44.0%, 53.3%, and 68.9% at 3, 5, 10, 15, 20, and 25\u00a0years, respectively. Long-term overall survival was favorable; however, malignancies accounted for 40% of deaths.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This nationwide survey updates the epidemiological and clinical characteristics of AIP in Japan. Patient numbers continue to rise, and the high cumulative risk of relapse underscores the need for second-line therapies beyond glucocorticoids.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00535-026-02438-w","type":"journal-article","created":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T08:42:04Z","timestamp":1779180124000},"page":"1502-1511","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 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