{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T03:34:27Z","timestamp":1791516867092,"version":"4.3.4"},"reference-count":38,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2019,10,26]],"date-time":"2019-10-26T00:00:00Z","timestamp":1572048000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2019,10,26]],"date-time":"2019-10-26T00:00:00Z","timestamp":1572048000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004330","name":"GlaxoSmithKline","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100004330","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arthritis Res Ther"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n              <jats:sec>\n                <jats:title>Objective<\/jats:title>\n                <jats:p>The objective of this randomized, placebo-controlled, double-blind, parallel group, trial was to assess the effect of ambrisentan on mean pulmonary arterial pressure (mPAP) in patients with systemic sclerosis (SSc) and mildly elevated pulmonary hypertension (PH).<\/jats:p>\n              <\/jats:sec>\n              <jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Thirty-eight SSc patients with mildly elevated mPAP at rest between 21 and 24\u2009mmHg and\/or &gt;\u200930\u2009mmHg during low-dose exercise were randomly assigned to treatment with either ambrisentan 5\u201310\u2009mg\/day or placebo. Right heart catheterization and further clinical parameters were assessed at baseline and after 6\u2009months. The primary endpoint was the difference of mPAP change at rest between groups.<\/jats:p>\n              <\/jats:sec>\n              <jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>After 6\u2009months, the two groups did not differ in the primary endpoint (ambrisentan mPAP \u2212\u20091\u2009\u00b1\u20096.4\u2009mmHg vs. placebo \u2212\u20090.73\u2009\u00b1\u20093.59\u2009mmHg at rest, <jats:italic>p<\/jats:italic>\u2009=\u20090.884). However, three patients from the placebo group but none of the ambrisentan group progressed to SSc-associated pulmonary arterial hypertension. Furthermore, ambrisentan treatment showed significant improvements in the secondary endpoints cardiac index (CI) and pulmonary vascular resistance (PVR) at rest (CI 0.36\u2009\u00b1\u20090.66\u2009l\/min\/m<jats:sup>2<\/jats:sup> vs. \u2212\u20090.31\u2009\u00b1\u20090.71\u2009l\/min\/m<jats:sup>2<\/jats:sup>, <jats:italic>p<\/jats:italic>\u2009=\u20090.010; PVR \u2212\u20090.70\u2009\u00b1\u20090.78\u00a0WU vs. 0.01\u2009\u00b1\u20090.71\u00a0WU, <jats:italic>p<\/jats:italic>\u2009=\u20090.012) and during exercise (CI 0.7\u2009\u00b1\u20090.81\u2009l\/min\/m<jats:sup>2<\/jats:sup> vs. \u2212\u20090.45\u2009\u00b1\u20091.36\u2009l\/min\/m<jats:sup>2<\/jats:sup>, <jats:italic>p<\/jats:italic>\u2009=\u20090.015; PVR \u2212\u20090.84\u2009\u00b1\u20090.48\u00a0WU vs. \u2212\u20090.0032\u2009\u00b1\u20090.34\u00a0WU, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001).<\/jats:p>\n              <\/jats:sec>\n              <jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>This is the first randomized, double-blind, placebo-controlled study testing the effect of ambrisentan in patients with mildly elevated mPAP and\/or exercise PH. The primary endpoint change in mPAP did only tendentially improve in the ambrisentan group, but the significant improvement of other hemodynamic parameters points to a possible benefit of ambrisentan and will be helpful to design future trials.<\/jats:p>\n              <\/jats:sec>\n              <jats:sec>\n                <jats:title>Trial registration<\/jats:title>\n                <jats:p>www.ClinicalTrials.gov, unique identifier NCT: <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/clinicaltrials.gov\/ct2\/show\/NCT02290613\">NCT02290613<\/jats:ext-link>, registered 14<jats:sup>th<\/jats:sup> of November 2014.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13075-019-1981-0","type":"journal-article","created":{"date-parts":[[2019,10,26]],"date-time":"2019-10-26T14:04:36Z","timestamp":1572098676000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":58,"title":["Early treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure associated with systemic sclerosis: a randomized, controlled, double-blind, parallel group study (EDITA study)"],"prefix":"10.1186","volume":"21","author":[{"given":"Zixuan","family":"Pan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto M.","family":"Marra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Benjamin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina A.","family":"Eichstaedt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norbert","family":"Blank","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"Bossone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Cittadini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerry","family":"Coghlan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher P.","family":"Denton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Distler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Egenlauf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satenik","family":"Harutyunova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Panagiota","family":"Xanthouli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanns-Martin","family":"Lorenz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekkehard","family":"Gr\u00fcnig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,10,26]]},"reference":[{"issue":"2","key":"1981_CR1","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1164\/rccm.200806-953OC","volume":"179","author":"Robin Condliffe","year":"2009","unstructured":"Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, et al. 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Pulm Circ. 2013;3(4):916-25","journal-title":"Pulmonary Circulation"}],"container-title":["Arthritis Research &amp; Therapy"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-019-1981-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s13075-019-1981-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13075-019-1981-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,10,24]],"date-time":"2020-10-24T23:19:16Z","timestamp":1603581556000},"score":1,"resource":{"primary":{"URL":"https:\/\/arthritis-research.biomedcentral.com\/articles\/10.1186\/s13075-019-1981-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10,26]]},"references-count":38,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2019,12]]}},"alternative-id":["1981"],"URL":"https:\/\/doi.org\/10.1186\/s13075-019-1981-0","relation":{},"ISSN":["1478-6362"],"issn-type":[{"value":"1478-6362","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10,26]]},"assertion":[{"value":"12 June 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 August 2019","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 October 2019","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"A written informed consent was obtained from all patients in accordance with the Declaration of Helsinki. The approval of the ethics committee of the Medical Faculty Heidelberg was obtained (AFmo-407\/2014).","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"ZP, AMM, CAE, EB, AC, CF, and PX declare that they have no competing interests. NBe has received speaker honoraria from Actelion pharmaceuticals, not related to this study. NoB has received speaker honoraria from Actelion pharmaceuticals, not related to this study. GC reports research support, consultancy fees, and speaker honoraria from Actelion pharmaceuticals. CPD reports speaker honoraria from Actelion pharmaceuticals. OD reports research support and consultancy fees from Actelion pharmaceuticals. BE reports speaker honoraria from Actelion pharmaceuticals. SH reports speaker honoraria from Actelion pharmaceuticals. HML reports speaker and consultancy fees from Actelion pharmaceuticals. EG has received speaker honoraria and advisory board fees from Actelion pharmaceuticals.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"217"}}