{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T22:30:54Z","timestamp":1784413854813,"version":"3.55.0"},"reference-count":73,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T00:00:00Z","timestamp":1693872000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pharmaceuticals"],"abstract":"<jats:p>Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to diverse groups: (1) pulmonary arterial hypertension (PAH), (2) PH due to left heart disease, (3) secondary PH due to lung disease and\/or hypoxia and (4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis. Therefore, early diagnosis should be established. A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase-five inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs, e.g., sotatercept, have been intensively investigated in clinical trials. We aim to review the literature on recent advances in the treatment strategy and prognosis of patients with PAH-CTD. In this manuscript, we discuss the mechanism of action of PAH-specific drugs and new agents and the latest research conducted on PAH-CTD patients.<\/jats:p>","DOI":"10.3390\/ph16091252","type":"journal-article","created":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T09:46:20Z","timestamp":1693907180000},"page":"1252","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases"],"prefix":"10.3390","volume":"16","author":[{"given":"Anna","family":"Smukowska-Gorynia","sequence":"first","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weronika","family":"Go\u015bciniak","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrycja","family":"Wo\u017aniak","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0509-6075","authenticated-orcid":false,"given":"Sylwia","family":"Iwa\u0144czyk","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karolina","family":"Jaxa-Kwiatkowska","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylwia","family":"S\u0142awek-Szmyt","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdalena","family":"Janus","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerzy","family":"Paluszkiewicz","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatiana","family":"Mularek-Kubzdela","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2 Street, 61-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,9,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1055\/s-0039-1685214","article-title":"Pulmonary Hypertension Associated with Connective Tissue Disease","volume":"40","author":"Fayed","year":"2019","journal-title":"Semin. 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