{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T08:05:24Z","timestamp":1789632324122,"version":"build-2803163510"},"reference-count":16,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T00:00:00Z","timestamp":1789603200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T00:00:00Z","timestamp":1789603200000},"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":["Internal Medicine Journal"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>Systemic sclerosis (scleroderma; SSc) patients can develop pulmonary arterial hypertension (PAH), which is a leading cause of death. We sought to evaluate severity, therapeutic strategy and survival of SSc\u2010PAH in the Australian Scleroderma Cohort Study (ASCS).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Among patients with 2013 American College of Rheumatology\/European League Against Rheumatism\u2010defined SSc, PAH was defined as mean pulmonary artery pressure (mPAP) \u226520\u2009mmHg, pulmonary arterial wedge pressure (PAWP) \u226415\u2009mmHg and pulmonary vascular resistance (PVR) &gt;2 WU. Characteristics of those with and without PAH and those with incident PAH in 2014\u20132020 versus 2007\u20132013 were compared using descriptive statistics. Survival was evaluated using the Kaplan\u2013Meier method and a multivariable Cox regression model.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 1612 patients, 71 (4.4%) had incident PAH prior to censoring on 13 February 2024.<\/jats:p>\n                    <jats:p>\n                      Significantly more patients received PDE5i monotherapy in the first 12 months after PAH diagnosis in the later epoch (30 (63.8%) vs 6 (25.0%),\n                      <jats:italic>P<\/jats:italic>\n                      =\u20090.002). Dual therapy (any endothelin reception antagonist (ERA) and any PDE5i) was more common in the later epoch (26 (55.3%) vs 4 (16.7%),\n                      <jats:italic>P<\/jats:italic>\n                      =\u20090.002). Overall survival of those diagnosed with PAH between 2007 and 2013 was 91.67%, 87.50% and 56.88% at 1, 3 and 5 years. Overall survival of those diagnosed between 2014 and 2020 was 100.00%, 77.62% and 48.98% at 1, 3 and 5 years (\n                      <jats:italic>P<\/jats:italic>\n                      =\u20090.414).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Despite increased use of dual therapy in the more recent epoch, we observed no significant improvement in overall survival in SSc\u2010PAH in our cohort. Our cohort's 1\u2010, 3\u2010 and 5\u2010year mortality is comparable to those reported for other contemporary SSc\u2010PAH cohorts.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/imj.70567","type":"journal-article","created":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T07:40:51Z","timestamp":1789630851000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Survival among patients with systemic sclerosis\u2010associated pulmonary arterial hypertension in the Australian scleroderma cohort study"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4973-8212","authenticated-orcid":false,"given":"Zoe","family":"Brown","sequence":"first","affiliation":[{"name":"St Vincent's Hospital, Melbourne Department of Rheumatology  Fitzroy Victoria Australia"},{"name":"The University of Melbourne Department of Medicine at St Vincent's Hospital  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dylan","family":"Hansen","sequence":"additional","affiliation":[{"name":"St Vincent's Hospital, Melbourne Department of Rheumatology  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen","family":"Morrisroe","sequence":"additional","affiliation":[{"name":"St Vincent's Hospital, Melbourne Department of Rheumatology  Fitzroy Victoria Australia"},{"name":"The University of Melbourne Department of Medicine at St Vincent's Hospital  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nava","family":"Ferdowsi","sequence":"additional","affiliation":[{"name":"St Vincent's Hospital, Melbourne Department of Rheumatology  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Ross","sequence":"additional","affiliation":[{"name":"St Vincent's Hospital, Melbourne Department of Rheumatology  Fitzroy Victoria Australia"},{"name":"The University of Melbourne Department of Medicine at St Vincent's Hospital  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy","family":"Stevens","sequence":"additional","affiliation":[{"name":"St Vincent's Hospital, Melbourne Department of Rheumatology  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryam","family":"Tabesh","sequence":"additional","affiliation":[{"name":"St Vincent's Hospital, Melbourne Department of Rheumatology  Fitzroy Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanne","family":"Sahhar","sequence":"additional","affiliation":[{"name":"Monash Health Department of Rheumatology  Clayton Victoria Australia"},{"name":"Department of Medicine Monash University  Clayton Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gene\u2010Siew","family":"Ngian","sequence":"additional","affiliation":[{"name":"Monash Health Department of Rheumatology  Clayton Victoria Australia"},{"name":"Department of Medicine Monash University  Clayton Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane","family":"Apostolopoulos","sequence":"additional","affiliation":[{"name":"Monash Health Department of Rheumatology  Clayton Victoria Australia"},{"name":"Department of Medicine Monash University  Clayton Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimti","family":"Kumar","sequence":"additional","affiliation":[{"name":"Royal Adelaide Hospital Rheumatology Unit  Adelaide South Australia Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer G.","family":"Walker","sequence":"additional","affiliation":[{"name":"Royal Adelaide Hospital Rheumatology Unit  Adelaide South Australia Australia"},{"name":"Rheumatology Unit Flinders Medical Centre (Adelaide)  Bedford Park South Australia Australia"},{"name":"Immunology, Allergy and Arthritis Department Flinders University  Bedford Park South Australia Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanna","family":"Proudman","sequence":"additional","affiliation":[{"name":"Royal Adelaide Hospital Rheumatology Unit  Adelaide South Australia Australia"},{"name":"Department of Medicine The University of Adelaide  Adelaide South Australia Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mandana","family":"Nikpour","sequence":"additional","affiliation":[{"name":"The University of Melbourne Department of Medicine at St Vincent's Hospital  Fitzroy Victoria Australia"},{"name":"University of Sydney Musculoskeletal Research Centre and School of Public Health University of Sydney  Sydney New South Wales Australia"},{"name":"Department of Rheumatology Royal Prince Alfred Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0056-2017"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.5301\/jsrd.5000256"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.40027"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00879-2022"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.30541"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13075-017-1341-x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2021.03.011"},{"key":"e_1_2_8_9_1","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1093\/rheumatology\/kead360","article-title":"Treatment strategies and survival of patients with connective tissue 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