{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T17:35:51Z","timestamp":1787592951365,"version":"build-2736575974"},"reference-count":51,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T00:00:00Z","timestamp":1769990400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T00:00:00Z","timestamp":1769990400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100020228","name":"Hamburgischen Stiftung f\u00fcr Wissenschaften, Entwicklung und Kultur Helmut und Hannelore Greve","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100020228","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["ME 3696\/3"],"award-info":[{"award-number":["ME 3696\/3"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000282","name":"Sir Jules Thorn Charitable Trust","doi-asserted-by":"publisher","award":["18JTA"],"award-info":[{"award-number":["18JTA"]}],"id":[{"id":"10.13039\/501100000282","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000395","name":"Royal College of Physicians","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000395","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background &amp; Aims<\/jats:title>\n                    <jats:p>Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) go along with reduced health\u2010related quality of life (HRQOL). Variant syndromes, that is, conditions with features of both PBC\/PSC and AIH, are associated with higher clinical complexity and worse prognosis. Studies on HRQOL in patients with variant syndromes are lacking. We aimed to provide large\u2010scale evidence addressing this gap.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We included adult patients with clinical diagnoses of autoimmune liver diseases across nine countries in a cross\u2010sectional study. We descriptively compared demographical, clinical and patient\u2010reported outcomes between the conditions and investigated whether additional AIH contributes to reduced HRQOL compared to the cholestatic liver disease alone. Further, we explored the role of fatigue, cirrhosis and depression severity regarding HRQOL.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      <jats:italic>N<\/jats:italic>\n                      \u2009=\u20091275 patients were included (PBC:\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009342, PBC\u2010AIH:\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009160, PSC:\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009305, PSC\u2010AIH:\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009121, AIH:\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009347). Patients with variant syndromes showed high rates of cirrhosis and increased depressive or anxiety symptoms. Additional AIH was associated with further reduction in physical and mental HRQOL in people with PSC (physical: \u0394\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u2009=\u20090.012,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.041; mental: \u0394\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u2009=\u20090.016,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.020), but not with PBC (physical: \u0394\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u2009=\u20090.008,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.081; mental: \u0394\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u2009=\u20090.001,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.609). Physical HRQOL was associated with higher age and fatigue, while mental HRQOL was associated with lower age, fatigue, and depression severity.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Patients with variant syndromes of autoimmune liver diseases show high physical and mental burden, with fatigue as the main contributor. Particularly PSC\u2010AIH goes along with more severely reduced HRQOL compared to the cholestatic liver disease alone, which is attributable to a higher symptom burden.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/liv.70526","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T10:12:37Z","timestamp":1770027157000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases\u2014A Cross\u2010Sectional Multicentre Study"],"prefix":"10.1111","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4031-9839","authenticated-orcid":false,"given":"Natalie","family":"Uhlenbusch","sequence":"first","affiliation":[{"name":"Department of Psychosomatic Medicine and Psychotherapy University Medical Centre Hamburg\u2010Eppendorf  Hamburg Germany"},{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rom\u00e9e J. A. L. M.","family":"Snijders","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology Radboud University Medical Centre  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meike","family":"Mund","sequence":"additional","affiliation":[{"name":"Martin Zeitz Centre for Rare Diseases University Medical Centre Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maciej K.","family":"Janik","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Hepatology, Transplantology and Internal Medicine Medical University of Warsaw  Warsaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piotr","family":"Milkiewicz","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Hepatology, Transplantology and Internal Medicine Medical University of Warsaw  Warsaw Poland"},{"name":"Translational Medicine Group Pomeranian Medical University  Szczecin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernd","family":"L\u00f6we","sequence":"additional","affiliation":[{"name":"Department of Psychosomatic Medicine and Psychotherapy University Medical Centre Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Kroll","sequence":"additional","affiliation":[{"name":"Martin Zeitz Centre for Rare Diseases University Medical Centre Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Raszeja\u2010Wyszomirska","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Hepatology, Transplantology and Internal Medicine Medical University of Warsaw  Warsaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessio","family":"Gerussi","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Division of Gastroenterology, Center for Autoimmune Liver Diseases, European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER) IRCCS Fondazione 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\/0000-0001-6102-1524","authenticated-orcid":false,"given":"Francesca","family":"Bolis","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Division of Gastroenterology, Center for Autoimmune Liver Diseases, European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER) IRCCS Fondazione San Gerardo Dei Tintori  Monza Italy"},{"name":"Department of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Cristoferi","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Division of Gastroenterology, Center for Autoimmune Liver Diseases, European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER) IRCCS Fondazione San Gerardo Dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3262-1998","authenticated-orcid":false,"given":"Pietro","family":"Invernizzi","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Division of Gastroenterology, Center for Autoimmune Liver Diseases, European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER) IRCCS Fondazione San Gerardo Dei Tintori  Monza Italy"},{"name":"Department of Medicine and Surgery University of Milano\u2010Bicocca  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Kovats","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  Debrecen Hungary"},{"name":"K\u00e1lm\u00e1n Laki Doctoral School, Faculty of Medicine University of Debrecen  Debrecen Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e1ria","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  Debrecen Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisbet","family":"Gr\u00f8nb\u00e6k","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Hepatology and Gastroenterology Aarhus University Hospital  Aarhus Denmark"},{"name":"Department of Medicine Regional Hospital Horsens  Horsens Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8998-7910","authenticated-orcid":false,"given":"Henning","family":"Gr\u00f8nb\u00e6k","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Hepatology and Gastroenterology Aarhus University Hospital  Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric T. T. L.","family":"Tjwa","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology Radboud University Medical Centre  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0046-887X","authenticated-orcid":false,"given":"Luise","family":"Aamann","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology Hvidovre University Hospital Copenhagen  Hvidovre Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henriette","family":"Ytting","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology Hvidovre University Hospital Copenhagen  Hvidovre Denmark"},{"name":"Department of Clinical Medicine, Faculty of Health Sciences University of Copenhagen  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0761-1333","authenticated-orcid":false,"given":"Vincenzo","family":"Ronca","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Liver Transplant and Hepatobiliary Unit, Queen Elizabeth Hospital, University Hospital of Birmingham NHS Foundation Trust &amp; Centre for Liver and Gastro Research, NIHR Biomedical Research Centre, Institute of Immunology and Immunotherapy University of Birmingham  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn","family":"Olsen","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Liver Transplant and Hepatobiliary Unit, Queen Elizabeth Hospital, University Hospital of Birmingham NHS Foundation Trust &amp; Centre for Liver and Gastro Research, NIHR Biomedical Research Centre, Institute of Immunology and Immunotherapy University of Birmingham  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ye H.","family":"Oo","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Liver Transplant and Hepatobiliary Unit, Queen Elizabeth Hospital, University Hospital of Birmingham NHS Foundation Trust &amp; Centre for Liver and Gastro Research, NIHR Biomedical Research Centre, Institute of Immunology and Immunotherapy University of Birmingham  Birmingham UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9871-4022","authenticated-orcid":false,"given":"Adriaan J.","family":"van der Meer","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology Erasmus University Medical Centre  Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o","family":"Madaleno","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Liver Disease Unit, Department of Internal Medicine Hospitais da Universidade de Coimbra, Unidade Local de Sa\u00fade de Coimbra  Coimbra Portugal"},{"name":"Liver Transplant Unit, Hospitais da Universidade de Coimbra, Unidade Local de Sa\u00fade de Coimbra, Faculty of Medicine University of Coimbra  Coimbra Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernardo","family":"Canh\u00e3o","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Liver Disease Unit, Department of Internal Medicine Hospitais da Universidade de Coimbra, Unidade Local de Sa\u00fade de Coimbra  Coimbra Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bastian","family":"Engel","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology Hannover Medical School  Hannover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alejandro","family":"Campos\u2010Murguia","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology Hannover Medical School  Hannover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Taubert","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology Hannover Medical School  Hannover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3678-5703","authenticated-orcid":false,"given":"\u00d6zg\u00fcr M.","family":"Koc","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology MUMC+  Maastricht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6248-8345","authenticated-orcid":false,"given":"Matthijs","family":"Kramer","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology MUMC+  Maastricht the Netherlands"},{"name":"GROW\u2014School for Oncology and Reproduction Maastricht University  Maastricht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9 A.","family":"Willemse","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Dutch Liver Patients Association  Amersfoort the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8823-3129","authenticated-orcid":false,"given":"Ansgar W.","family":"Lohse","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"I. Department of Medicine University Medical Centre Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8027-3073","authenticated-orcid":false,"given":"Joost P. H.","family":"Drenth","sequence":"additional","affiliation":[{"name":"European Reference Network on Hepatological Diseases (ERN RARE\u2010LIVER)  Hamburg Germany"},{"name":"Department of Gastroenterology and Hepatology Amsterdam UMC  Amsterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3070-8443","authenticated-orcid":false,"given":"Tom J. 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