دورية أكاديمية
Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases
| العنوان: | Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases |
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| المؤلفون: | Anna Smukowska-Gorynia, Weronika Gościniak, Patrycja Woźniak, Sylwia Iwańczyk, Karolina Jaxa-Kwiatkowska, Sylwia Sławek-Szmyt, Magdalena Janus, Jerzy Paluszkiewicz, Tatiana Mularek-Kubzdela |
| المصدر: | Pharmaceuticals, Vol 16, Iss 9, p 1252 (2023) |
| بيانات النشر: | MDPI AG, 2023. |
| سنة النشر: | 2023 |
| المجموعة: | LCC:Medicine LCC:Pharmacy and materia medica |
| مصطلحات موضوعية: | PAH CTD, novel drugs, therapy, treatment, prognosis, Medicine, Pharmacy and materia medica, RS1-441 |
| الوصف: | 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. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 1424-8247 |
| Relation: | https://www.mdpi.com/1424-8247/16/9/1252; https://doaj.org/toc/1424-8247 |
| DOI: | 10.3390/ph16091252 |
| URL الوصول: | https://doaj.org/article/19c1bfe6557d41159f0a194a24fd9e0b |
| رقم الأكسشن: | edsdoj.19c1bfe6557d41159f0a194a24fd9e0b |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 14248247 |
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| DOI: | 10.3390/ph16091252 |