{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T04:04:33Z","timestamp":1791345873410,"version":"4.3.1"},"reference-count":15,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,2,24]],"date-time":"2014-02-24T00:00:00Z","timestamp":1393200000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis Care &amp; Research"],"published-print":{"date-parts":[[2014,3]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)\u2013associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right\u2010sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure \u226525 mm Hg and pulmonary capillary wedge pressure \u226415 mm Hg without significant interstitial lung disease) were included in these analyses.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 131 SSc patients with incident PAH were followed for a mean \u00b1 SD of 2.0 \u00b1 1.4 years. The 1\u2010, 2\u2010, and 3\u2010year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age &gt;60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1\u20138.4), male sex (HR 3.9, 95% CI 1.1\u201313.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8\u201322.8), and diffusing capacity for carbon monoxide (DL<jats:sc>co<\/jats:sc>) &lt;39% predicted (HR 4.2, 95% CI 1.3\u201313.8) were significant predictors of mortality.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This is the largest study describing survival in patients with incident SSc\u2010associated PAH followed up at multiple SSc centers in the US who had undergone routine screening for PAH. The survival rates were better than those reported in other recently described SSc\u2010associated PAH cohorts. Severely reduced DL<jats:sc>co<\/jats:sc> and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.22121","type":"journal-article","created":{"date-parts":[[2013,8,27]],"date-time":"2013-08-27T16:42:54Z","timestamp":1377621774000},"page":"489-495","source":"Crossref","is-referenced-by-count":145,"title":["Survival and Predictors of Mortality in Systemic Sclerosis\u2010Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry"],"prefix":"10.1002","volume":"66","author":[{"given":"Lorinda","family":"Chung","sequence":"first","affiliation":[{"name":"Stanford University, Stanford, and VA Palo Alto Health Care System Palo Alto California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robyn T.","family":"Domsic","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bharathi","family":"Lingala","sequence":"additional","affiliation":[{"name":"Stanford University Stanford California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Firas","family":"Alkassab","sequence":"additional","affiliation":[{"name":"University of Massachusetts Worcester"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcy","family":"Bolster","sequence":"additional","affiliation":[{"name":"Medical University of South Carolina Charleston"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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