{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,23]],"date-time":"2026-05-23T10:04:47Z","timestamp":1779530687913,"version":"3.53.1"},"reference-count":18,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T00:00:00Z","timestamp":1757548800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T00:00:00Z","timestamp":1757548800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Hepatology Research"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>The Japan Primary Biliary Cholangitis Study Group (JPBCSG) has conducted nationwide surveys of primary biliary cholangitis (PBC) since 1980. Due to the progress in the management of PBC, a more comprehensive survey platform is desired.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The JPBCSG conducted the 17th survey of PBC by using an electronic data capture (EDC) system: The patients' clinical demography at diagnosis and the outcomes of newly and past registered cases (till the 16th) were entered jointly in the EDC system. A questionnaire for treatment response was included in the survey. Using the new platform, the trends in clinical demography and the long\u2010term prognosis of PBC were analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The percentage of male patients with PBC showed a significant increase from the period before the end of 2010 [Period 1, 12.4% (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a0772)] to the period after 2021 [Period 4, 20.6% (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a0345),\n                      <jats:italic>p\u00a0&lt;<\/jats:italic>\n                      \u00a00.001] (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a02579, in total). The mean age at diagnosis also rose significantly from 56.8\u00a0years old in Period 1 to 61.8\u00a0years in Period 4 (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0&lt;\u00a00.001). There were no significant changes in the prevalence of symptoms at diagnosis. In a multivariate Cox\u2010regression analysis, older age (hazard ratio [HR] 3.48,\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.001), female sex (HR 0.46,\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.046), total bilirubin (HR 3.84,\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.009), fluid retention (HR 5.69,\n                      <jats:italic>p\u00a0&lt;<\/jats:italic>\n                      \u00a00.001), and esophagogastric varices (HR 2.66,\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.015) were significantly associated with liver\u2010related mortality and liver transplantation.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>We have established the 2nd\u2010generation nationwide survey of PBC in Japan. The survey results revealed not only demographical trends in Japanese patients with PBC but also risk factors for their prognosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hepr.70035","type":"journal-article","created":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T10:20:57Z","timestamp":1757586057000},"page":"50-59","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical Characteristics and Long\u2010Term Prognosis of Primary Biliary Cholangitis in Japan: Results of the 2nd\u2010Generation Nationwide Survey"],"prefix":"10.1111","volume":"56","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-2849-9057","authenticated-orcid":false,"given":"Yuki","family":"Kugiyama","sequence":"first","affiliation":[{"name":"NHO Nagasaki Medical Center  Omura Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4489-9102","authenticated-orcid":false,"given":"Masanori","family":"Abe","sequence":"additional","affiliation":[{"name":"Department of Community and General Medicine Ehime University Graduate School of Medicine  Matsuyama Japan"},{"name":"Department of Gastroenterology and Metabology Ehime University Graduate School of Medicine  Matsuyama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3158-5318","authenticated-orcid":false,"given":"Tadashi","family":"Namisaki","sequence":"additional","affiliation":[{"name":"Nara Medical University Hospital  Kashiwara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5243-8544","authenticated-orcid":false,"given":"Hitoshi","family":"Yoshiji","sequence":"additional","affiliation":[{"name":"Nara Medical University Hospital  Kashiwara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazumichi","family":"Abe","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Fukushima Medical University School of Medicine  Fukushima Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiromasa","family":"Ohira","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Fukushima Medical University School of Medicine  Fukushima Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7843-3138","authenticated-orcid":false,"given":"Ryosaku","family":"Shirahashi","sequence":"additional","affiliation":[{"name":"Dokkyo Medical University Saitama Medical Center  Koshigaya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keiji","family":"Yokoyama","sequence":"additional","affiliation":[{"name":"Fukuoka University Hospital  Fukuoka 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Medical University Ibaraki Medical Center Inashiki\u2010gun  Kasumigaura Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9216-3186","authenticated-orcid":false,"given":"Tadashi","family":"Ikegami","sequence":"additional","affiliation":[{"name":"Tokyo Medical University Ibaraki Medical Center Inashiki\u2010gun  Kasumigaura Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shiho","family":"Miyase","sequence":"additional","affiliation":[{"name":"Kumamoto Shinto General Hospital  Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-8869-7673","authenticated-orcid":false,"given":"Toshiaki","family":"Nakano","sequence":"additional","affiliation":[{"name":"The Third Department of Internal Medicine Kansai Medical University  Osaka 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