Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases
Patients with connective tissue diseases (CTD) may develop pulmonary hypertension (PH) belonging to different groups: (1) pulmonary arterial hypertension (PAH), (2) PH due to left heart disease, (3) PH secondary to lung disease and/or hypoxia (CTD patients mostly develop interstitial lung disease), and (4) chronic thromboembolic pulmonary hypertension (CTEPH), especially in the setting of antiphospholipid syndrome (APS). A specific treatment for PAH-CTD is currently available and recommended (solid arrows): prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase-5 inhibitors (PDE5i: sildenafil, tadalafil) and endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Two other pathways are under intensive investigation (dashed arrows): (1) affecting BMPR2: trapping the ligands of TGF-β and reducing the activity of ACTRIIA (sotatercept, KER-012) or inhibiting PDGFR (imatinib, seralutinib), (2) inhibiting the serotonin pathway by blocking the serotonin-producing enzyme tryptophan hydroxylase 1 (rodatristat ethyl).
Publication
Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases. (2023) Anna Smukowska-Gorynia, et al. Pharmaceuticals (Basel). 2023 Sep;16(9):1252. Figure: F1.| Organism | Group | Word | Match | Source | NCBI Symbol | NCBI ID |
|---|---|---|---|---|---|---|
| Homo sapiens | Primates | CTD | CTD | ncbigene_symbol | CTD | 1283 |
| Homo sapiens | Primates | APS | APS | ncbigene_synonym | KLK3 | 354 |
| Homo sapiens | Primates | APS | APS | ncbigene_synonym | SH2B2 | 10603 |
| Homo sapiens | Primates | ERA | ERA | ncbigene_synonym | ESR1 | 2099 |
| Homo sapiens | Primates | ERA | ERA | ncbigene_synonym | ERAL1 | 26284 |
| Homo sapiens | Primates | PAH | PAH | ncbigene_symbol | PAH | 5053 |
| Homo sapiens | Primates | BMPR2 | BMPR2 | ncbigene_symbol | BMPR2 | 659 |
| Homo sapiens | Primates | ACTR | ACTR | ncbigene_synonym | NCOA3 | 8202 |
| Homo sapiens | Primates | trap) | TRAP | ncbigene_symbol | TRAP | 100187907 |
| Homo sapiens | Primates | trap) | TRAP | ncbigene_synonym | ACP5 | 54 |
| Homo sapiens | Primates | trap) | TRAP | ncbigene_synonym | CD40LG | 959 |
| Homo sapiens | Primates | trap) | TRAP | ncbigene_synonym | TRAF2 | 7186 |
| Homo sapiens | Primates | trap) | TRAP | ncbigene_synonym | TRRAP | 8295 |
| Homo sapiens | Primates | trap) | TRAP | ncbigene_synonym | TDRD7 | 23424 |
| Homo sapiens | Primates | trap) | TRAP | ncbigene_synonym | SCYL1 | 57410 |
| Homo sapiens | Primates | PDGFR | PDGFR | ncbigene_synonym | PDGFRB | 5159 |
| Homo sapiens | Primates | PDGFR | PDGFR | famplex_relations | PDGFRA | 5156 |
| Word | Match | MeSH | Name | ChEBI |
|---|---|---|---|---|
| PAH | p-Aminohippuric Acid | mesh:D010130 | p-aminohippuric acid | chebi:104011 |
| NO | NA | |||
| Serotonin | Serotonin | mesh:D012701 | serotonin | chebi:28790 |
| tryptofan | NA |
Disease mentions
| Word | Match | MeSH | Name | DOID |
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