{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T11:56:20Z","timestamp":1791201380596,"version":"4.2.0"},"reference-count":19,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T00:00:00Z","timestamp":1779840000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T00:00:00Z","timestamp":1779840000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP24ek0109744"],"award-info":[{"award-number":["JP24ek0109744"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100012045","name":"Kawano Masanori Memorial Public Interest Incorporated Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012045","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["24K18883"],"award-info":[{"award-number":["24K18883"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["25K19298"],"award-info":[{"award-number":["25K19298"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["24K02437"],"award-info":[{"award-number":["24K02437"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["25K11149"],"award-info":[{"award-number":["25K11149"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100016925","name":"Takeda Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100016925","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Hepatology Research"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>Pediatric primary sclerosing cholangitis (PSC) is a rare progressive cholestatic liver disease that often presents diagnostic challenges owing to overlapping clinical and histological features with autoimmune hepatitis (AIH). We hypothesized that anti\u2010integrin \u03b1v\u03b26 autoantibodies may serve as a noninvasive biomarker to differentiate pediatric PSC from AIH and monitor disease activity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed serum samples from 65 patients with pediatric\u2010onset PSC, 39 with AIH, and 66 controls. Anti\u2010integrin \u03b1v\u03b26 autoantibodies were quantified using enzyme\u2010linked immunosorbent assay. Functional activity of the autoantibodies was assessed using a solid\u2010phase fibronectin\u2010binding inhibition assay.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Anti\u2010integrin \u03b1v\u03b26 autoantibody levels were significantly higher in pediatric\u2010onset PSC than in AIH and controls. The sensitivity and specificity for distinguishing PSC were 63.9% (95% confidence interval [CI]: 51.4\u201374.8) and 97.1% (95% CI: 91.9\u201399.2), respectively. Higher levels were observed in PSC with inflammatory bowel disease than without (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.0069) and in PSC\u2013AIH overlap compared with AIH alone (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.0018). In one case, rising autoantibody levels preceded the clinical transition from AIH to PSC\u2013AIH overlap. Levels declined during remission and after liver transplantation; however, increased again upon recurrence. Functional assays demonstrated that autoantibodies from patients with pediatric\u2010onset PSC inhibited binding of integrin \u03b1v\u03b26 to fibronectin.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Anti\u2010integrin \u03b1v\u03b26 autoantibodies may serve as a noninvasive clinically informative biomarker for distinguishing pediatric\u2010onset PSC from AIH and reflecting longitudinal changes in disease activity, including posttransplant recurrence. These findings support their potential clinical utility in the diagnosis and longitudinal assessment of pediatric\u2010onset PSC.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hepr.70211","type":"journal-article","created":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T03:56:58Z","timestamp":1779940618000},"page":"1447-1456","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Anti\u2010Integrin \u03b1v\u03b26 Autoantibodies as Diagnostic and Monitoring Biomarkers for Pediatric\u2010Onset Primary Sclerosing Cholangitis"],"prefix":"10.1111","volume":"56","author":[{"given":"Yukako","family":"Maeda","sequence":"first","affiliation":[{"name":"Department of Pediatrics Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuichiro","family":"Umetsu","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hepatology and Gastroenterology Saiseikai Yokohama\u2010shi Tobu Hospital  Yokohama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6663-6656","authenticated-orcid":false,"given":"Eitaro","family":"Hiejima","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Kyoto University Graduate School of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitsuyoshi","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Juntendo University Faculty of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8677-8195","authenticated-orcid":false,"given":"Itaru","family":"Iwama","sequence":"additional","affiliation":[{"name":"Department of Hepatology and Gastroenterology Saitama Children's Medical Center  Saitama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1179-8903","authenticated-orcid":false,"given":"Takeshi","family":"Kimura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Graduate School of Medicine The University of Osaka  Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonoko","family":"Kondo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Faculty of Medicine Kagawa University  Kagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1973-4376","authenticated-orcid":false,"given":"Ayano","family":"Inui","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hepatology and Gastroenterology Saiseikai Yokohama\u2010shi Tobu Hospital  Yokohama 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