{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T21:24:44Z","timestamp":1774301084632,"version":"3.50.1"},"reference-count":39,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2013,7,11]],"date-time":"2013-07-11T00:00:00Z","timestamp":1373500800000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2013,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Pulmonary arterial hypertension (<jats:styled-content style=\"fixed-case\">PAH<\/jats:styled-content>) is a major cause of mortality in systemic sclerosis (<jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content>). There is emerging evidence that screening may enable the earlier detection and treatment of <jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">PAH<\/jats:styled-content>, and thereby improve survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>We undertook a systematic review to evaluate the performance of current screening algorithms in <jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">PAH<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We searched the Medline and <jats:styled-content style=\"fixed-case\">EMBASE<\/jats:styled-content> databases to 31 <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>arch 2012. We selected studies if they applied a screening algorithm to consecutively enrolled <jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content> patients not known to have <jats:styled-content style=\"fixed-case\">PAH<\/jats:styled-content>; <jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">PAH<\/jats:styled-content> had to be confirmed on right heart catheterisation (<jats:styled-content style=\"fixed-case\">RHC<\/jats:styled-content>). The performance of each screening algorithm and the methodological quality of each study was evaluated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Nine studies met the inclusion criteria with a total intent\u2010to\u2010screen population of 3504 participants. In studies of patients with prevalent disease, the positive predictive value (<jats:styled-content style=\"fixed-case\">PPV<\/jats:styled-content>) of screening for <jats:styled-content style=\"fixed-case\">PAH<\/jats:styled-content> was 20.4\u201387.0%. In studies of patients with incident disease, the <jats:styled-content style=\"fixed-case\">PPV<\/jats:styled-content> of screening for <jats:styled-content style=\"fixed-case\">PAH<\/jats:styled-content> was 20.0\u201330.7%. The <jats:styled-content style=\"fixed-case\">PPV<\/jats:styled-content> of algorithms using echocardiography alone, or in combination with other tests, was comparable. No study enabled an accurate determination of negative predictive value, sensitivity or specificity of the screening algorithm as only patients who screened positive underwent confirmatory testing with <jats:styled-content style=\"fixed-case\">RHC<\/jats:styled-content>. The optimal timing and frequency of repeat screening is unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The low to moderate <jats:styled-content style=\"fixed-case\">PPV<\/jats:styled-content> of current screening algorithms, coupled with the inability to determine accurately the negative predictive value, sensitivity and specificity, suggests that there is a need to validate further these algorithms before making recommendations regarding screening for <jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">PAH<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/imj.12167","type":"journal-article","created":{"date-parts":[[2013,4,24]],"date-time":"2013-04-24T11:07:04Z","timestamp":1366801624000},"page":"751-760","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Performance of screening algorithms in systemic sclerosis\u2010related pulmonary arterial hypertension: a systematic review"],"prefix":"10.1111","volume":"43","author":[{"given":"V.","family":"Thakkar","sequence":"first","affiliation":[{"name":"Department of Rheumatology St Vincent's Hospital Melbourne"},{"name":"Department of Rheumatology Liverpool Hospital  Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W. 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