Buchumschlag

Treatment of pulmonary arterial hypertension in connective tissue disease

Pulmonary arterial hypertension (PAH) is a group of distinct disorders that includes idiopathic PAH (IPAH), familial PAH and PAH associated with other conditions (APAH) such as connective tissue disease (CTD-APAH) or congenital heart disease. PAH is characterized by increased pulmonary arterial pres...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
1. Verfasser: Grünig, Ekkehard (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 2012
In: Drugs
Year: 2012, Jahrgang: 72, Heft: 8, Pages: 1039-1056
ISSN:1179-1950
DOI:10.2165/11633390-000000000-00000
Online-Zugang:Verlag, Volltext: http://dx.doi.org/10.2165/11633390-000000000-00000
Verlag, Volltext: https://link.springer.com/article/10.2165/11633390-000000000-00000
Volltext
Verfasserangaben:Ekkehard Grünig
Beschreibung
Zusammenfassung:Pulmonary arterial hypertension (PAH) is a group of distinct disorders that includes idiopathic PAH (IPAH), familial PAH and PAH associated with other conditions (APAH) such as connective tissue disease (CTD-APAH) or congenital heart disease. PAH is characterized by increased pulmonary arterial pressure and pulmonary vascular resistance. If left untreated, PAH can lead to right heart failure and premature death. CTD-APAH rep-resents an important clinical subgroup of APAH that has a higher risk of death than IPAH. The European treatment guidelines advocate the use of PAH-targeted therapies including bosentan, ambrisentan, sildenafil, inhaled iloprost, intravenous epoprostenol (I-A recommendations), tadalafil or treprostinil (I-B recommendations) for patients in WHO functional class II-III. Not all randomized clinical studies of the approved PAH-targeted therapies have included patients with CTD-APAH. The purpose of this review is to describe the clinical characteristics of CTD-APAH and discuss the approved pharmacological treatments, with a focus on data specific to this subgroup where possible.
Beschreibung:First online: 10 December 2012
Gesehen am 22.06.2018
Beschreibung:Online Resource
ISSN:1179-1950
DOI:10.2165/11633390-000000000-00000

Ähnliche Einträge