{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T08:45:45Z","timestamp":1792053945862,"version":"4.4.0"},"reference-count":0,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T00:00:00Z","timestamp":1792022400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T00:00:00Z","timestamp":1792022400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"abstract":"\n Objective<\/jats:title>\n Real\u2010world initial treatment strategies in SSc\u2010PAH remain incompletely described. We aimed to characterise these strategies across haemodynamic thresholds and risk strata and evaluate outcome associations.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n We analysed treatment\u2010na\u00efve SSc\u2010PAH patients in EUSTAR (2000\u20132021) fulfilling the 2022 haemodynamic definition. Initial therapy comprised treatment initiated within 4 months of RHC. As\u2010treated (AT) analyses were primary and intention\u2010to\u2010treat (ITT) analyses supportive. Outcomes were all\u2010cause mortality and 36\u2010month PAH progression.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n Among 301 patients, 198 (66%) initiated therapy and 170 maintained therapy for \u22653 months. Therapy was initiated in 31% with milder haemodynamic impairment (mPAP 21\u201324 mmHg and\/or PVR 2\u20133 WU) versus 79% fulfilling the previous haemodynamic definition; combination therapy increased from 14% to 67% across low\u2010 to high\u2010risk strata. Over a median 3.9 years, 125 (42%) died and 85\/227 (37%) progressed. Adjusted ITT analyses showed no association with either outcome, whereas adjusted AT analyses showed lower mortality risk with sustained monotherapy and combination therapy (HR 0.50, 95% CI 0.27\u20130.92 and HR 0.40, 95% CI 0.18\u20130.88, respectively) and lower PAH progression risk with sustained initial therapy (HR 0.37, 95% CI 0.18\u20130.76). In the milder haemodynamic subgroup, the mortality estimate was directionally consistent but imprecise.<\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n Initial treatment strategies reflected haemodynamic severity and baseline risk, while variation suggested potential implementation gaps. Sustained initial therapy was associated with lower risks of mortality and PAH progression. Contrasting ITT and AT estimates highlight challenges in observational treatment\u2013outcome comparisons. Findings in the milder haemodynamic subgroup support dedicated prospective studies.<\/jats:p>\n \n \n \n image<\/jats:alt-text>\n <\/jats:graphic>\n <\/jats:boxed-text>\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/art.70372","type":"journal-article","created":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T08:06:58Z","timestamp":1792051618000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Initial treatment strategies and outcomes across haemodynamic thresholds and risk stratification in incident systemic sclerosis\u2013associated pulmonary arterial hypertension: a EUSTAR analysis"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-0321-375X","authenticated-orcid":false,"given":"Hilde Jenssen","family":"Bj\u00f8rkekj\u00e6r","sequence":"first","affiliation":[{"name":"Department of Rheumatology, Hospital of Southern Norway Kristiansand Norway"},{"name":"Institute of Clinical Medicine University of Oslo Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cosimo","family":"Bruni","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University Hospital Zurich University of Zurich Zurich Switzerland"},{"name":"Department of Experimental and Clinical Medicine University of Florence Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaspar","family":"Broch","sequence":"additional","affiliation":[{"name":"Department of Cardiology Oslo University Hospital, Rikshospitalet Oslo Norway"},{"name":"Institute for Experimental Medical Research, KG Jebsen Center University of Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cathrine","family":"Brunborg","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine University of Oslo Oslo Norway"},{"name":"Oslo Centre for Biostatistics and Epidemiology, Research Support Services Oslo University Hospital \u2010 Rikshospitalet Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia E","family":"Carreira","sequence":"additional","affiliation":[{"name":"Department of Rheumatology 12 de Octubre University Hospital Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Air\u00f2","sequence":"additional","affiliation":[{"name":"UOC Reumatologia ed Immunologia Clinica, Spedali Civili di Brescia, Scleroderma UNIT, Piazzale Spedali Civili 1, 25123 Brescia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen Pilar","family":"Sime\u00f3n\u2010Aznar","sequence":"additional","affiliation":[{"name":"Systemic Autoimmune Diseases Unit, Department of Internal Medicine University Vall d'Hebron Hospital Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Elise","family":"Truchetet","sequence":"additional","affiliation":[{"name":"Rheumatology Department Bordeaux University Hospital Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Giollo","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Medicine University of Verona Verona Italy"},{"name":"Rheumatology Unit, Department of Medicine \u2010 DIMED University and Hospital of Padua Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Balbir\u2010Gurman","sequence":"additional","affiliation":[{"name":"Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion\u2010Institute of Technology Haifa Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mickael","family":"Martin","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Poitiers University Hospital Poitiers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3975-8938","authenticated-orcid":false,"given":"Christopher P","family":"Denton","sequence":"additional","affiliation":[{"name":"Centre for Rheumatology and Connective Tissue Diseases University College London Division of Medicine and Royal Free Hospital London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3189-5516","authenticated-orcid":false,"given":"Armando","family":"Gabrielli","sequence":"additional","affiliation":[{"name":"Fondazione di Medicina Molecolare e Terapia Cellulare, Universit\u00e0 Politecnica delle Marche Ancona Italy"},{"name":"Hiller Research Center, University Hospital D\u00fcsseldorf Medical Faculty of Heinrich Heine University D\u00fcsseldorf Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8528-2283","authenticated-orcid":false,"given":"Francesco","family":"Del Galdo","sequence":"additional","affiliation":[{"name":"Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM Leeds UK"},{"name":"NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust Leeds UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2266-9907","authenticated-orcid":false,"given":"Madelon C.","family":"Vonk","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Radboud Universiteit Nijmegen Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e5vard","family":"Fretheim","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine University of Oslo Oslo Norway"},{"name":"Lillehammer Hospital for Rheumatic Diseases Lillehammer Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helle","family":"Bitter","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Hospital of Southern Norway Kristiansand Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d8yvind","family":"Midtvedt","sequence":"additional","affiliation":[{"name":"Department of Rheumatology Oslo University Hospital, Rikshospitalet Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arne K.","family":"Andreassen","sequence":"additional","affiliation":[{"name":"Department of Cardiology Oslo University Hospital, Rikshospitalet Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sverre","family":"H\u00f8ie","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine University of Oslo Oslo Norway"},{"name":"Department of Cardiology, Hospital of Southern Norway Arendal Norway"},{"name":"ProCardio Center for Innovation, Clinic of Heart\u2010, Lung\u2010 and Vascular Diseases Oslo University Hospital, Rikshospitalet Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0807-7139","authenticated-orcid":false,"given":"Yoshiya","family":"Tanaka","sequence":"additional","affiliation":[{"name":"First Department of Internal Medicine University of Occupational and Environmental Health Kitakyushu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriela","family":"Riemekasten","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology University of L\u00fcbeck L\u00fcbeck Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulf","family":"M\u00fcller\u2010Ladner","sequence":"additional","affiliation":[{"name":"Dept of Rheumatology and Clinical Immunology, Justus\u2010Liebig University Giessen, Campus Kerckhoff Bad Nauheim Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Matucci\u2010Cerinic","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), & Inflammation, fibrosis and ageing initiative (INFLAGE), IRCCS San Raffaele Hospital Milan Italy"},{"name":"University Vita Salute San Raffaele Milan Italy"},{"name":"Inflammation Fibrosis and Ageing Initiative (INFLAGE), Division of Genetics and Cell Biology IRCCS San Raffaele Scientific Institute Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5410-5807","authenticated-orcid":false,"given":"Ivan","family":"Castellv\u00ed","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Institut de Recerca Sant Pau (IR Sant Pau), Hospital de la Santa Creu i Sant Pau Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elise","family":"Siegert","sequence":"additional","affiliation":[{"name":"Rheumatology Charit\u00e9 University Hospital Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Hachulla","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical Immunology, Referral Centre for rare systemic autoimmune diseases North of France, North\u2010West, Mediterranean and Guadeloupe (CeRAINOM), CHU Lille, Univ. Lille, Inserm, U1286 France"},{"name":"INFINITE \u2010 Institute for Translational Research in Inflammation, F\u201059000 Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d8yvind","family":"Molberg","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine University of Oslo Oslo Norway"},{"name":"Department of Rheumatology Oslo University Hospital, Rikshospitalet Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2413-1959","authenticated-orcid":false,"given":"Oliver","family":"Distler","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University Hospital Zurich University of Zurich Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna\u2010Maria","family":"Hoffmann\u2010Vold","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University Hospital Zurich University of Zurich Zurich Switzerland"},{"name":"Department of Rheumatology Oslo University Hospital, Rikshospitalet Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the EUSTAR collaborators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,10,15]]},"container-title":["Arthritis & Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/art.70372","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/art.70372","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T08:06:59Z","timestamp":1792051619000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/art.70372"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,15]]},"references-count":0,"alternative-id":["10.1002\/art.70372"],"URL":"https:\/\/doi.org\/10.1002\/art.70372","archive":["Portico"],"relation":{},"ISSN":["2326-5191","2326-5205"],"issn-type":[{"value":"2326-5191","type":"print"},{"value":"2326-5205","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,15]]},"assertion":[{"value":"2026-03-26","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-09-28","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-10-15","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"art.70372"}}