{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T05:22:12Z","timestamp":1792732932388,"version":"4.5.0"},"reference-count":37,"publisher":"BMJ","issue":"3","license":[{"start":{"date-parts":[[2023,4,4]],"date-time":"2023-04-04T00:00:00Z","timestamp":1680566400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Scleroderma and Related Disorders"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Several studies described the cross-sectional characteristics of systemic sclerosis patients and coexisting primary biliary cholangitis, but longitudinal prognostic data are lacking.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aims:<\/jats:title>\n                    <jats:p>To describe the systemic sclerosis\u2013primary biliary cholangitis phenotype, including baseline characteristics and outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>We performed a multicentre the European Scleroderma Trials and Research Group study of systemic sclerosis patients with primary biliary cholangitis or with primary biliary cholangitis\u2013specific antibodies, matched with systemic sclerosis controls free from hepatobiliary involvement matched for disease duration and cutaneous subset. Data were recorded at baseline and at the last available visit.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>A total of 261 patients were enrolled (115 primary biliary cholangitis\u2013systemic sclerosis, 161 systemic sclerosis). At baseline, systemic sclerosis\u2013primary biliary cholangitis patients had a higher prevalence of anti-centromere antibodies ( p\u2009=\u20090.0023) and a lower prevalence of complete absence of digital ulcers. The milder vascular involvement was confirmed at follow-up when crucial differences emerged in the percentage of patients experiencing digital ulcers; a significantly higher number of patients who never experienced digital ulcers were observed among primary biliary cholangitis\u2013systemic sclerosis patients ( p\u2009=\u20090.0015). Moreover, a greater incidence of pulmonary arterial hypertension ( p\u2009&lt;\u20090.001) and of conduction blocks ( p\u2009=\u20090.0256) was observed in systemic sclerosis patients without primary biliary cholangitis. Patients with primary biliary cholangitis had higher levels of liver enzymes at baseline than systemic sclerosis patients; a significant decrease in liver enzymes was observed at follow-up. Out of 18 patients with cholangitis, one received a liver transplant at follow-up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>Our data show that systemic sclerosis\u2013primary biliary cholangitis exhibit a mild systemic sclerosis and primary biliary cholangitis phenotype with outcomes being in general favourable.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/23971983231155948","type":"journal-article","created":{"date-parts":[[2023,4,5]],"date-time":"2023-04-05T00:12:42Z","timestamp":1680653562000},"page":"210-220","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":8,"title":["Systemic sclerosis and primary biliary cholangitis: Longitudinal data to determine the outcomes"],"prefix":"10.1136","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4141-6937","authenticated-orcid":false,"given":"Gemma","family":"Lepri","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5241-1918","authenticated-orcid":false,"given":"Paolo","family":"Air\u00f2","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Distler","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University Hospital Zurich, University of Zurich, Z\u00fcrich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7021-2541","authenticated-orcid":false,"given":"Kristofer","family":"Andr\u00e9asson","sequence":"additional","affiliation":[{"name":"Section of Rheumatology, Department of Clinical Sciences, Lund University, Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7706-9346","authenticated-orcid":false,"given":"Yolanda","family":"Braun-Moscovici","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Hachulla","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, H\u00f4pital Claude Huriez, Lille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Balbir-Gurman","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen","family":"De Langhe","sequence":"additional","affiliation":[{"name":"ERN ReCONNET, Division of Rheumatology, University Hospitals Leuven, Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simona","family":"Rednic","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Emergency County Teaching Hospital, University of Medicine and Pharmacy Iuliu Hatieganu, Cluj-Napoca, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Ingegnoli","sequence":"additional","affiliation":[{"name":"Clinical Rheumatology Unit, ASST Pini-CTO, Department of Clinical Sciences and Community Health, Universit\u00e0 degli Studi di Milano, Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edoardo","family":"Rosato","sequence":"additional","affiliation":[{"name":"Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Groseanu","sequence":"additional","affiliation":[{"name":"Carol Davila University of Medicine and Pharmacy, Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruxandra","family":"Ionescu","sequence":"additional","affiliation":[{"name":"Carol Davila University of Medicine and Pharmacy, Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Bellando-Randone","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5012-0540","authenticated-orcid":false,"given":"Liudmila","family":"Garzanova","sequence":"additional","affiliation":[{"name":"Laboratory of Microcirculation and Inflammation, VA Nasonova Institute of Rheumatology, Moscow, Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Beretta","sequence":"additional","affiliation":[{"name":"Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, La Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore di Milano Policlinico, Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8326-7904","authenticated-orcid":false,"given":"Chiara","family":"Bellocchi","sequence":"additional","affiliation":[{"name":"Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, La Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore di Milano Policlinico, Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergey","family":"Moiseev","sequence":"additional","affiliation":[{"name":"Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pavel","family":"Novikov","sequence":"additional","affiliation":[{"name":"Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russian Federation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iulia","family":"Szabo","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Emergency County Teaching Hospital, University of Medicine and Pharmacy Iuliu Hatieganu, Cluj-Napoca, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorota","family":"Krasowska","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin, Lublin, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronica","family":"Codullo","sequence":"additional","affiliation":[{"name":"Rheumatology, Policlinico San Matteo, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich A.","family":"Walker","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Universit\u00e4tsspital Basel, Basel, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chrysoula","family":"Manolaraki","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Universit\u00e4tsspital Basel, Basel, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serena","family":"Guiducci","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Elise","family":"Truchetet","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Bordeaux University Hospital, Bordeaux, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0474-5344","authenticated-orcid":false,"given":"Florenzo","family":"Iannone","sequence":"additional","affiliation":[{"name":"Rheumatology Unit \u2013 DETO, School of Medicine, University of Bari, Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Tofani","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2813-2083","authenticated-orcid":false,"given":"Cosimo","family":"Bruni","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy"},{"name":"Department of Rheumatology, University Hospital Zurich, University of Zurich, Z\u00fcrich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanessa","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Ghent University Hospital and Department of Internal Medicine, Ghent University, Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4292-3589","authenticated-orcid":false,"given":"Giovanna","family":"Cuomo","sequence":"additional","affiliation":[{"name":"Department of Precision of Medicine, University of Campania \u2013 L. Vanvitelli, Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Krusche","sequence":"additional","affiliation":[{"name":"Division of Rheumatology and Systemic Inflammatory Diseases, University Hospital Hamburg-Eppendorf (UKE), Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Matucci-Cerinic","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy"},{"name":"Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, La Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore di Milano Policlinico, Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yannick","family":"Allanore","sequence":"additional","affiliation":[{"name":"Rheumatology, Cochin Hospital, APHP, Paris Cit\u00e9 University, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2023,4,4]]},"reference":[{"key":"bibr1-23971983231155948","doi-asserted-by":"publisher","DOI":"10.5301\/jsrd.5000249"},{"issue":"2","key":"bibr2-23971983231155948","first-page":"202","volume":"15","author":"LeRoy EC","year":"1988","journal-title":"J Rheumatol"},{"key":"bibr3-23971983231155948","doi-asserted-by":"publisher","DOI":"10.5301\/jsrd.5000212"},{"key":"bibr4-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2019.102452"},{"key":"bibr5-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1002\/art.40906"},{"key":"bibr6-23971983231155948","doi-asserted-by":"publisher","DOI":"10.5301\/jsrd.5000213"},{"key":"bibr7-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1002\/art.38098"},{"key":"bibr8-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1007\/s00296-006-0207-3"},{"key":"bibr9-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2013.05.002"},{"key":"bibr10-23971983231155948","first-page":"973","volume":"99","author":"Milbradt W.","year":"1934","journal-title":"Dermatol Monatsschr"},{"key":"bibr11-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.3.5717.258"},{"key":"bibr12-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2017.10.004"},{"key":"bibr13-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1111\/liv.14378"},{"key":"bibr14-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-3-1"},{"key":"bibr15-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1177\/2397198318802763"},{"key":"bibr16-23971983231155948","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.110167"},{"key":"bibr17-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1016\/j.dld.2009.01.014"},{"key":"bibr18-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-010-1586-0"},{"key":"bibr19-23971983231155948","doi-asserted-by":"crossref","first-page":"862","DOI":"10.20452\/pamw.3583","volume":"11","author":"Wielosz E","year":"2016","journal-title":"Pol Arch Med Wewn"},{"key":"bibr20-23971983231155948","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.090340"},{"key":"bibr21-23971983231155948","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.090815"},{"key":"bibr22-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1016\/j.bpg.2013.06.016"},{"key":"bibr23-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1155\/2011\/976427"},{"key":"bibr24-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1136\/gut.2005.075002"},{"key":"bibr25-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1177\/2397198318757699"},{"key":"bibr26-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2021.102842"},{"key":"bibr27-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/37.1.39"},{"key":"bibr28-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1007\/s12072-019-09984-x"},{"issue":"237","key":"bibr29-23971983231155948","first-page":"75","volume":"62","author":"Powell FC","year":"1987","journal-title":"Q J Med"},{"key":"bibr30-23971983231155948","doi-asserted-by":"publisher","DOI":"10.3109\/s10165-012-0607-z"},{"key":"bibr31-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1515\/cclm-2019-0655"},{"key":"bibr32-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1136\/ard.60.11.1046"},{"key":"bibr33-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinre.2013.04.005"},{"key":"bibr34-23971983231155948","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2021.625763"},{"key":"bibr35-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.14103"},{"key":"bibr36-23971983231155948","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2018.06.009"},{"issue":"8","key":"bibr37-23971983231155948","first-page":"1488","volume":"32","author":"Miyawaki S","year":"2005","journal-title":"J Rheumatol"}],"container-title":["Journal of Scleroderma and Related Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/23971983231155948","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/23971983231155948","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/23971983231155948","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T11:52:57Z","timestamp":1777981977000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/23971983231155948"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,4]]},"references-count":37,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,10]]}},"alternative-id":["10.1177\/23971983231155948"],"URL":"https:\/\/doi.org\/10.1177\/23971983231155948","relation":{},"ISSN":["2397-1983","2397-1991"],"issn-type":[{"value":"2397-1983","type":"print"},{"value":"2397-1991","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,4]]}}}