{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T14:59:46Z","timestamp":1784818786133,"version":"3.55.0"},"reference-count":82,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T00:00:00Z","timestamp":1646092800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>In systemic sclerosis (SSc) mortality is mainly linked to lung involvement which is characterized by interstitial lung disease (ILD) and pulmonary hypertension (PH). In SSc, PH may be due to different etiologies, including ILD, chronic thromboembolic disease, pulmonary veno-occlusive disease, and pulmonary arterial hypertension (PAH). The main tool to screen PAH is transthoracic echocardiography (TTE), which has a sensitivity of 90%, even if definitive diagnosis should be confirmed by right heart catheterization (RHC). The radiological evaluation (i.e., HRTC) plays an important role in defining the possible causes and in monitoring the evolution of lung damage. For PAH, identifying individuals who have borderline elevation of pulmonary arterial pressure needs to be appropriately managed and followed. In the past few years, the strategy for the management of PAH has significantly evolved and new trials are underway to test other therapies. This review provides an overview of the tools to evaluate PAH in SSc patients and on treatment options for these patients.<\/jats:p>","DOI":"10.3390\/diagnostics12030616","type":"journal-article","created":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T08:17:03Z","timestamp":1646122623000},"page":"616","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["An Overview of Different Techniques for Improving the Treatment of Pulmonary Hypertension Secondary in Systemic Sclerosis Patients"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8990-859X","authenticated-orcid":false,"given":"Barbara","family":"Ruaro","sequence":"first","affiliation":[{"name":"Department of Pulmonology, Cattinara Hospital, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0864-9766","authenticated-orcid":false,"given":"Francesco","family":"Salton","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Cattinara Hospital, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1061-9507","authenticated-orcid":false,"given":"Elisa","family":"Baratella","sequence":"additional","affiliation":[{"name":"Department of Radiology, Cattinara Hospital, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7284-5016","authenticated-orcid":false,"given":"Paola","family":"Confalonieri","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Cattinara Hospital, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pietro","family":"Geri","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Cattinara Hospital, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riccardo","family":"Pozzan","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Cattinara Hospital, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9110-3029","authenticated-orcid":false,"given":"Chiara","family":"Torregiani","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Cattinara Hospital, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1737-5776","authenticated-orcid":false,"given":"Roberta","family":"Bulla","sequence":"additional","affiliation":[{"name":"Department of Life Sciences, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4791-768X","authenticated-orcid":false,"given":"Marco","family":"Confalonieri","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Cattinara Hospital, University of Trieste, 34149 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Matucci-Cerinic","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze, 50139 Florence, Italy"},{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3361-4909","authenticated-orcid":false,"given":"Michael","family":"Hughes","sequence":"additional","affiliation":[{"name":"Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-Under-Lyne OL6 9RW, UK"},{"name":"Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Salford Royal NHS Foundation Trust, Manchester M1 1AA, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,3,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e000782","DOI":"10.1136\/rmdopen-2018-000782","article-title":"Systemic sclerosis: State of the art on clinical practice guidelines","volume":"4","author":"Smith","year":"2019","journal-title":"RMD Open"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Ruaro, B., Confalonieri, M., Matucci-Cerinic, M., Salton, F., Confalonieri, P., Santagiuliana, M., Citton, G., Baratella, E., and Bruni, C. 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