Pulmonary Hypertension Associated with Connective Tissue Disease.
Connective tissue disease
Diagnosis
Epidemiology
Outcomes
Pulmonary hypertension
Journal
Rheumatic diseases clinics of North America
ISSN: 1558-3163
Titre abrégé: Rheum Dis Clin North Am
Pays: United States
ID NLM: 8708093
Informations de publication
Date de publication:
Aug 2024
Aug 2024
Historique:
medline:
29
6
2024
pubmed:
29
6
2024
entrez:
28
6
2024
Statut:
ppublish
Résumé
Pulmonary hypertension (PH), a syndrome characterized by elevated pulmonary pressures, commonly complicates connective tissue disease (CTD) and is associated with increased morbidity and mortality. The incidence of PH varies widely between CTDs; patients with systemic sclerosis are most likely to develop PH. Several different types of PH can present in CTD, including PH related to left heart disease and respiratory disease. Importantly, CTD patients are at risk for developing pulmonary arterial hypertension, a rare form of PH that is associated with high morbidity and mortality. Future therapies targeting pulmonary vascular remodeling may improve outcomes for patients with this devastating disease.
Identifiants
pubmed: 38942575
pii: S0889-857X(24)00031-0
doi: 10.1016/j.rdc.2024.03.005
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
359-379Informations de copyright
Copyright © 2024 Elsevier Inc. All rights reserved.
Déclaration de conflit d'intérêts
Disclosure Consultancies: Actelion, Acceleron, Bayer, United Therapeutics. Funding to institution: Actelion, United Therapeutics. NHLBI: U01HL125175 (Co-PI); R01HL134905 (Co-I); R01HL11490 (Co-I). DOD: PR191839 (PI). The clinical trial (NCT04797286) referenced in the article is supported by DOD grant PR191839 (PI-Mathai).