{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T23:17:40Z","timestamp":1787872660017,"version":"build-2784847793"},"reference-count":151,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T00:00:00Z","timestamp":1727654400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Precapillary pulmonary hypertension (PH) is a significant complication of systemic sclerosis (SSc). It represents one of the leading causes of morbidity and mortality, correlating with a significantly dismal prognosis and quality of life. Despite advancements in the management of patients with pulmonary arterial hypertension associated with SSc (SSc-PAH), no significant improvement has been reported in survival of patients with precapillary SSc-PH associated with extensive lung parenchyma disease. International expert consensus and guidelines for the management of PH recommend annual screening of SSc patients for early detection of pre-capillary PH. The implementation of screening algorithms capable of identifying patients with a high likelihood of developing PH could help limit unnecessary right-heart catheterization procedures and prevent significant delay in diagnosis. Furthermore, early initiation of up-front combination targeted therapy in patients with PAH has shown increase in survival rates, indicating that timely and aggressive medical therapy is key for stabilizing and even improving functional class, hemodynamic parameters and 6 min walking distance (6MWD) in this population. Further research is warranted into the benefit of PAH-targeted therapies in patients with PH associated with lung disease. Lastly, we discuss the potential role of immunosuppression using biologic agents in the therapeutic management of precapillary PH in SSc patients.<\/jats:p>","DOI":"10.3390\/jcm13195834","type":"journal-article","created":{"date-parts":[[2024,9,30]],"date-time":"2024-09-30T07:19:37Z","timestamp":1727680777000},"page":"5834","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Towards a Better Prognosis in Patients with Systemic Sclerosis-Related Pulmonary Arterial Hypertension: Recent Developments and Perspectives"],"prefix":"10.3390","volume":"13","author":[{"given":"Maria","family":"Boutel","sequence":"first","affiliation":[{"name":"Fourth Department of Internal Medicine, Hippokration University Hospital, School of Medicine, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-8180-2273","authenticated-orcid":false,"given":"Athanasia","family":"Dara","sequence":"additional","affiliation":[{"name":"Fourth Department of Internal Medicine, Hippokration University Hospital, School of Medicine, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3180-7280","authenticated-orcid":false,"given":"Alexandra","family":"Arvanitaki","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy\u2019s and St Thomas\u2019s NHS Foundation Trust, Imperial College, London SW3 6NP, UK"},{"name":"First Department of Cardiology, AHEPA University Hospital, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, 54636 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cleopatra","family":"Deuteraiou","sequence":"additional","affiliation":[{"name":"Fourth Department of Internal Medicine, Hippokration University Hospital, School of Medicine, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Mytilinaiou","sequence":"additional","affiliation":[{"name":"Fourth Department of Internal Medicine, Hippokration University Hospital, School of Medicine, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0364-1642","authenticated-orcid":false,"given":"Theodoros","family":"Dimitroulas","sequence":"additional","affiliation":[{"name":"Fourth Department of Internal Medicine, Hippokration University Hospital, School of Medicine, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1080\/1744666X.2023.2171988","article-title":"Understanding and Managing Cardiac Involvement in Systemic Sclerosis","volume":"19","author":"Moysidou","year":"2023","journal-title":"Expert. 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