{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T07:30:06Z","timestamp":1791876606656,"version":"4.3.4"},"reference-count":52,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2023,2,1]],"date-time":"2023-02-01T00:00:00Z","timestamp":1675209600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/100000050","name":"The National Institutes of Health\/National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01 HL114910"],"award-info":[{"award-number":["R01 HL114910"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"The National Institutes of Health\/National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["U01HL125175-01"],"award-info":[{"award-number":["U01HL125175-01"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"name":"The National Institutes of Health\/National Heart, Lung, and Blood Institute","award":["R01 HL114910"],"award-info":[{"award-number":["R01 HL114910"]}]},{"name":"The National Institutes of Health\/National Heart, Lung, and Blood Institute","award":["U01HL125175-01"],"award-info":[{"award-number":["U01HL125175-01"]}]}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>To date, it remains unclear whether recent changes in the management of patients with systemic sclerosis\u2013associated pulmonary hypertension (SSc-PH) have improved survival.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To describe a cohort of patients with SSc-PH and compare their characteristics and survival between the last two decades.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with SSc-PH prospectively enrolled in the Johns Hopkins Pulmonary Hypertension Center Registry were grouped into two cohorts based on the date of diagnostic right heart catheterization: cohort A included patients whose disease was diagnosed between 1999 and 2010, and cohort B included those whose disease was diagnosed between 2010 and 2021. Patients\u2019 characteristics were compared between the two cohorts.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Measurements and Main Results<\/jats:title>\n                    <jats:p>Of 504 patients with SSc-PH distributed almost equally between the two cohorts, 308 (61%) had World Symposium on Pulmonary Hypertension group 1, 43 (9%) had group 2, and 151 (30%) had group 3 disease. Patients with group 1 disease in cohort B had significantly better clinical and hemodynamic characteristics at diagnosis, were more likely to receive upfront combination pulmonary arterial hypertension therapy, and had a nearly 4-year increase in median transplant-free survival in univariable analysis than those in cohort A (P\u2009&amp;lt;\u20090.01). Improved transplant-free survival was still observed after adjusting for patients\u2019 baseline characteristics. In contrast, for group 2 or 3 patients with SSc-PH, there were no differences in baseline clinical, hemodynamic, or survival characteristics between the two cohorts.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This is the largest single-center study that compares clinical characteristics of patients with SSc-PH between the last two decades. Transplant-free survival has improved significantly for those with group 1 disease over the last decade, possibly secondary to earlier detection and better therapeutic management. Conversely, those with group 2 or 3 disease continue to have dismal prognosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1164\/rccm.202204-0731oc","type":"journal-article","created":{"date-parts":[[2022,9,29]],"date-time":"2022-09-29T13:45:58Z","timestamp":1664459158000},"page":"312-322","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":52,"title":["Improved Survival for Patients with Systemic Sclerosis\u2013associated Pulmonary Arterial Hypertension: The Johns Hopkins Registry"],"prefix":"10.1093","volume":"207","author":[{"given":"Hussein J.","family":"Hassan","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Naranjo","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine 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