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Approximately one in 10 will develop PAH<\/jats:styled-content> during their lifetime. These patients have a worse prognosis than those with PAH<\/jats:styled-content> due to other causes. The most common clinical feature of SSc\u2010PAH<\/jats:styled-content> in the early stages is non\u2010specific exercise intolerance that can be erroneously attributed to other manifestations of SSc. Screening provides an opportunity for early identification of SS<\/jats:styled-content>c\u2010PAH<\/jats:styled-content> and prompt initiation of therapies with the potential to improve quality of life and survival. International guidelines recommend annual transthoracic D<\/jats:styled-content>oppler echocardiography (TTE<\/jats:styled-content>), but TTE<\/jats:styled-content> has limitations. The tricuspid regurgitant jet required for estimating the systolic pulmonary artery pressure is absent in up to 39% of patients, including a proportion with PAH<\/jats:styled-content>. This has prompted a move to new screening algorithms that are less dependent on TTE<\/jats:styled-content>. Not all pulmonary hypertension (PH<\/jats:styled-content>) in patients with SS<\/jats:styled-content>c is PAH<\/jats:styled-content>. Other causes include PH<\/jats:styled-content> secondary to left heart disease, interstitial lung disease\u2010related PH, chronic thromboembolic PH<\/jats:styled-content> and pulmonary veno\u2010occlusive disease. With the advent of evidence\u2010based therapies, including newer agents such as macitentan, riociguat and selexipag, the establishment of centres with expertise in PAH<\/jats:styled-content> and the focus on early detection, there has been considerable improvement in survival. The role of anti\u2010coagulation for SSc\u2010PAH<\/jats:styled-content> has been the subject of a recent meta\u2010analysis of nine observational studies that suggests it may confer a survival benefit, but to date, there have been no randomised controlled trials to confirm this.<\/jats:p>","DOI":"10.1111\/imj.12691","type":"journal-article","created":{"date-parts":[[2015,3,3]],"date-time":"2015-03-03T10:49:33Z","timestamp":1425379773000},"page":"248-254","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Prospects for improving outcomes in systemic sclerosis\u2010related pulmonary hypertension"],"prefix":"10.1111","volume":"45","author":[{"given":"V.","family":"Thakkar","sequence":"first","affiliation":[{"name":"Department of Rheumatology Liverpool Hospital Sydney New South Wales Australia"},{"name":"School of Medicine University of Western Sydney Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Nikpour","sequence":"additional","affiliation":[{"name":"Department of Rheumatology St Vincent's Hospital Melbourne Melbourne Victoria Australia"},{"name":"Department of Medicine The University of Melbourne Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. 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ASIG) for pulmonary arterial hypertension in systemic sclerosis","volume":"32","author":"Hao YJ","year":"2014","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_2_3_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.37838"},{"key":"e_1_2_3_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.30423"},{"key":"e_1_2_3_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.34501"},{"key":"e_1_2_3_27_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1209657"}],"container-title":["Internal Medicine Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fimj.12691","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/imj.12691","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T15:35:36Z","timestamp":1695569736000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/imj.12691"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,3]]},"references-count":26,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2015,3]]}},"alternative-id":["10.1111\/imj.12691"],"URL":"https:\/\/doi.org\/10.1111\/imj.12691","archive":["Portico"],"relation":{},"ISSN":["1444-0903","1445-5994"],"issn-type":[{"value":"1444-0903","type":"print"},{"value":"1445-5994","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,3]]},"assertion":[{"value":"2014-11-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2014-11-27","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2015-03-03","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}