{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T18:43:01Z","timestamp":1772131381311,"version":"3.50.1"},"reference-count":31,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2017,5,12]],"date-time":"2017-05-12T00:00:00Z","timestamp":1494547200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["1 U54 GM104940"],"award-info":[{"award-number":["1 U54 GM104940"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["1P306M106392\u201001A1"],"award-info":[{"award-number":["1P306M106392\u201001A1"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2017,10]]},"abstract":"\nABSTRACT<\/jats:styled-content>\n<\/jats:title>Background and objective<\/jats:title>Systemic sclerosis (SSc<\/jats:styled-content>) is a complex autoimmune disease commonly associated with pulmonary hypertension (PH<\/jats:styled-content>). When associated with elevated pulmonary artery wedge pressure (PAWP<\/jats:styled-content>), pulmonary artery pressure (PAP<\/jats:styled-content>) is either in\u2010proportion (post\u2010capillary PH<\/jats:styled-content>) or higher than expected (combined PH<\/jats:styled-content>) relative to the increased PAWP<\/jats:styled-content>.<\/jats:p><\/jats:sec>Methods<\/jats:title>Patients from the PHAROS<\/jats:styled-content> registry (a prospective observational cohort of SSc\u2010PH<\/jats:styled-content> patients) who had mean PAP<\/jats:styled-content>\u2009\u2265\u200925 and PAWP<\/jats:styled-content>\u2009>\u200915 on right heart catheterization were stratified based on diastolic pressure gradient (DPG<\/jats:styled-content>). Kaplan\u2013Meier analysis was performed to compare survival and PH<\/jats:styled-content>\u2010related hospitalization. Baseline factors were compared between patients dying and those who survived using Cox regression analysis.<\/jats:p><\/jats:sec>Results<\/jats:title>A total of 59 patients were included, of whom 21 (36%) patients were classified as combined PH<\/jats:styled-content> and 38 (64%) had post\u2010capillary PH<\/jats:styled-content>. No baseline characteristics were significantly different between the two groups. There were no differences in survival or PH<\/jats:styled-content>\u2010related hospitalization between the groups. The only baseline factor independently associated with death was lower 6\u2010min walk distance (6MWD<\/jats:styled-content>) (hazard ratio (HR<\/jats:styled-content>): 1.33 per 25\u2009m decrease, 95% CI<\/jats:styled-content>: 1.11\u20131.59, P<\/jats:italic>\u2009=\u20090.002). PH<\/jats:styled-content>\u2010specific medications were started during follow\u2010up in significantly more patients in the combined PH<\/jats:styled-content> group compared with the post\u2010capillary group (86% vs 50%, P<\/jats:italic>\u2009=\u20090.01).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>Outcomes were similar between SSc<\/jats:styled-content> patients with post\u2010capillary PH<\/jats:styled-content> and combined pre\u2010 and post\u2010capillary PH<\/jats:styled-content>. 6MWD<\/jats:styled-content> at baseline can predict risk for death in SSc<\/jats:styled-content> patients with PH<\/jats:styled-content> and an elevated PAWP<\/jats:styled-content>. More patients with combined PH<\/jats:styled-content> were started on PH<\/jats:styled-content>\u2010specific medications, and the clinical benefit of treating this subgroup specifically in SSc<\/jats:styled-content> patients needs further exploration.<\/jats:p><\/jats:sec>","DOI":"10.1111\/resp.13067","type":"journal-article","created":{"date-parts":[[2017,5,13]],"date-time":"2017-05-13T03:04:20Z","timestamp":1494644660000},"page":"1386-1392","source":"Crossref","is-referenced-by-count":9,"title":["Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: O<\/scp>bservations from the PHAROS<\/scp> cohort"],"prefix":"10.1111","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5125-1801","authenticated-orcid":false,"given":"Matthew R.","family":"Lammi","sequence":"first","affiliation":[{"name":"Pulmonary\/Critical Care and Allergy\/Immunology Louisiana State University Health Sciences Center New Orleans Louisiana USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lesley A.","family":"Saketkoo","sequence":"additional","affiliation":[{"name":"Section of Pulmonary Diseases, Critical Care and Environmental Medicine Tulane University School of Medicine New Orleans Louisiana USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica K.","family":"Gordon","sequence":"additional","affiliation":[{"name":"Department of Rheumatology Hospital for Special Surgery New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paula","family":"Lauto","sequence":"additional","affiliation":[{"name":"Pulmonary\/Critical Care and Allergy\/Immunology Louisiana State University Health Sciences Center New Orleans Louisiana USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karen","family":"Fagan","sequence":"additional","affiliation":[{"name":"Pulmonary and Critical Care University of South Alabama Mobile Alabama USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Virginia D.","family":"Steen","sequence":"additional","affiliation":[{"name":"Division of Rheumatology Georgetown University Washington DC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"on behalf of the PHAROS Investigators","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,5,12]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.029"},{"key":"e_1_2_7_3_1","first-page":"269","article-title":"Assessment of left and right ventricular diastolic function in patients with systemic sclerosis","volume":"66","author":"Ciurzynski MBP","year":"2008","journal-title":"Kardiol. 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