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- W4386439623 abstract "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." @default.
- W4386439623 created "2023-09-06" @default.
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- W4386439623 date "2023-09-05" @default.
- W4386439623 modified "2023-10-17" @default.
- W4386439623 title "Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases" @default.
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- W4386439623 doi "https://doi.org/10.3390/ph16091252" @default.
- W4386439623 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/37765060" @default.
- W4386439623 hasPublicationYear "2023" @default.
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