{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T08:57:08Z","timestamp":1784883428636,"version":"3.55.0"},"reference-count":23,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T00:00:00Z","timestamp":1761091200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T00:00:00Z","timestamp":1761091200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["UEG Journal"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>The European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER) is a Europe\u2010wide network for centers of excellence in the management of rare liver diseases. We aimed to evaluate the current diagnostic and therapeutic trends of primary biliary cholangitis (PBC).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Prospective data of PBC cases diagnosed from 2017 to March 2024 were extracted from the R\u2010LIVER registry of ERN\u2010RARE LIVER. Cases without two follow\u2010ups within 24\u00a0months were excluded from the treatment analysis. Biochemical response according to Toronto criteria and normalization of alkaline phosphatase (ALP) values after 12\u00a0months of Ursodeoxycholic Acid (UDCA) were evaluated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>This study included 327 incident cases from six centers. Median age was 56\u00a0years, 89.3% were female. At the time of diagnosis, median values of ALP were 1.37 x ULN, and median bilirubin was 0.49 x ULN. Transient elastography (TE) was performed in 230 patients (70.3%) at baseline; median liver stiffness was 6.2\u00a0kPa. Out of 316 subjects, treatment with UDCA was started in 312 patients (98.7%); 246 (85.1%) achieved ALP values\u00a0&lt;\u00a01.67 x ULN at 12\u00a0months. Normalization of ALP values occurred in 143 subjects (49.5%) at 12\u00a0months. Among 43 patients with inadequate response, 18 (41.9%) were treated with second\u2010line therapies, and had worse liver biochemistry at baseline.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In the current era, patients with PBC are diagnosed at an early stage using non\u2010invasive methods and are almost all treated with UDCA. The biochemical response rate is 85.1%, but the use of second\u2010line therapies for inadequate responders remains suboptimal.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ueg2.70134","type":"journal-article","created":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T16:30:17Z","timestamp":1761150617000},"page":"1955-1963","source":"Crossref","is-referenced-by-count":3,"title":["Trends in Primary Biliary Cholangitis: Prospective Cohort Study From the European Reference Network Registry (R\u2010LIVER)"],"prefix":"10.1002","volume":"13","author":[{"given":"A.","family":"Gerussi","sequence":"first","affiliation":[{"name":"Centre for Autoimmune Liver Diseases and Division of Gastroenterology European Reference Network (ERN) RARE\u2010LIVER Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"},{"name":"Department of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Nofit","sequence":"additional","affiliation":[{"name":"Centre for Autoimmune Liver Diseases and Division of Gastroenterology European Reference Network (ERN) RARE\u2010LIVER Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"},{"name":"Department of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8771-5428","authenticated-orcid":false,"given":"D. P.","family":"Bernasconi","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Kroll","sequence":"additional","affiliation":[{"name":"Department of Medicine University Medical Centre Hamburg\u2010Eppendorf (UKE)  Hamburg Germany"},{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Gro\u00df","sequence":"additional","affiliation":[{"name":"Department of Medicine University Medical Centre Hamburg\u2010Eppendorf (UKE)  Hamburg Germany"},{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"Schregel","sequence":"additional","affiliation":[{"name":"Department of Medicine University Medical Centre Hamburg\u2010Eppendorf (UKE)  Hamburg Germany"},{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Marini","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9846-7474","authenticated-orcid":false,"given":"L.","family":"Cristoferi","sequence":"additional","affiliation":[{"name":"Centre for Autoimmune Liver Diseases and Division of Gastroenterology European Reference Network (ERN) RARE\u2010LIVER Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Malinverno","sequence":"additional","affiliation":[{"name":"Centre for Autoimmune Liver Diseases and Division of Gastroenterology European Reference Network (ERN) RARE\u2010LIVER Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3662-4010","authenticated-orcid":false,"given":"M.","family":"Papp","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Division of Gastroenterology Department of Internal Medicine Faculty of Medicine University of Debrecen  Hungary Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7075-8464","authenticated-orcid":false,"given":"G.","family":"Dalekos","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Medicine and Research Laboratory of Internal Medicine National Expertise Center of Greece in Autoimmune Liver Diseases General University Hospital of Larissa  Larissa Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1978-4602","authenticated-orcid":false,"given":"E.","family":"Rigopoulou","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Medicine and Research Laboratory of Internal Medicine National Expertise Center of Greece in Autoimmune Liver Diseases General University Hospital of Larissa  Larissa Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1941-0336","authenticated-orcid":false,"given":"M. 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