{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T09:44:58Z","timestamp":1790675098524,"version":"4.1.0"},"reference-count":50,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2016,1,25]],"date-time":"2016-01-25T00:00:00Z","timestamp":1453680000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Association des Scl\u00e9rodermiques de France"},{"name":"Soci\u00e9t\u00e9 Nationale Fran\u00e7aise de M\u00e9decine Interne"},{"DOI":"10.13039\/100014846","name":"Groupe Pasteur Mutualit\u00e9","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100014846","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Institut Servier"},{"DOI":"10.13039\/100004330","name":"GlaxoSmithKline","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100004330","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2016,2]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTDs). This study aimed to investigate the clinical and hemodynamic characteristics and survival of anti\u2013U1 RNP\u2013positive patients with CTD\u2010associated PAH, with a focus on systemic sclerosis (SSc)\u2013associated PAH.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We implemented a prospective database that included patients with CTD\u2010associated PAH for whom there were clinical, autoantibody, and mortality data. We compared clinical and hemodynamic characteristics to anti\u2013U1 RNP antibody status. We then assessed whether anti\u2013U1 RNP antibodies could be a prognostic factor in CTD\u2010associated PAH with a focus on SSc\u2010associated PAH.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We studied a total of 342 patients with CTD\u2010associated PAH, of whom 36 (11%) were anti\u2013U1 RNP antibody positive. Anti\u2013U1 RNP\u2013positive patients were younger and less functionally impaired than were anti\u2013U1 RNP\u2013negative patients in CTD\u2010 and SSc\u2010associated PAH. Hemodynamic parameters were similar in anti\u2013U1 RNP\u2013positive and anti\u2013U1 RNP\u2013negative patients. In CTD\u2010associated PAH, anti\u2013U1 RNP positivity was associated with decreased mortality in univariable analysis (hazard ratio 0.34 [95% confidence interval 0.18\u20130.65], <jats:italic>P<\/jats:italic> &lt; 0.001). In multivariable analysis, anti\u2013U1 RNP positivity was also associated with decreased mortality (hazard ratio 0.44 [95% confidence interval 0.20\u20130.97], <jats:italic>P<\/jats:italic>\u2009=\u20090.043) independently of age, sex, functional parameters, lung involvement, and hemodynamic parameters. Results were similar in SSc\u2010associated PAH, although the association between anti\u2013U1 RNP positivity and survival did not reach significance in univariable (hazard ratio 0.47 [95% confidence interval 0.22\u20131.02], <jats:italic>P<\/jats:italic>\u2009=\u20090.055) and multivariable (hazard ratio 0.47 [95% confidence interval 0.20\u20131.11], <jats:italic>P<\/jats:italic>\u2009=\u20090.085) analyses.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Anti\u2013U1 RNP positivity was associated with distinct clinical characteristics and survival in CTD\u2010 and SSc\u2010associated PAH. While hemodynamic parameters were similar in anti\u2013U1 RNP\u2013positive and anti\u2013U1 RNP\u2013negative patients, our results suggest that anti\u2013U1 RNP positivity could be a factor protecting against mortality in CTD\u2010 and SSc\u2010associated PAH.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.39432","type":"journal-article","created":{"date-parts":[[2015,9,28]],"date-time":"2015-09-28T12:48:11Z","timestamp":1443444491000},"page":"484-493","source":"Crossref","is-referenced-by-count":85,"title":["Characteristics and Survival of Anti\u2013U1 RNP Antibody\u2013Positive Patients With Connective Tissue Disease\u2013Associated Pulmonary Arterial Hypertension"],"prefix":"10.1002","volume":"68","author":[{"given":"Vincent","family":"Sobanski","sequence":"first","affiliation":[{"name":"Royal Free Hospital and University College London, London, UK, and Universit\u00e9 de Lille, Centre National de R\u00e9f\u00e9rence de la Scl\u00e9rodermie Syst\u00e9mique, H\u00f4pital Claude Huriez, EA2686, INSERM U995, Lille Inflammation Research International Centre, and F\u00e9d\u00e9ration Hospitalo\u2010Universitaire Immune\u2010Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT)  Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Giovannelli","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Lille, F\u00e9d\u00e9ration Hospitalo\u2010Universitaire IMMINeNT, and Centre Hospitalier R\u00e9gional Universitaire de Lille  Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernadette M.","family":"Lynch","sequence":"additional","affiliation":[{"name":"Royal Free Hospital and University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin E.","family":"Schreiber","sequence":"additional","affiliation":[{"name":"Royal Free Hospital and University College London  London 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