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Drugs
Erschienen in: Drugs 8/2012

01.05.2012 | Review Article

Treatment of Pulmonary Arterial Hypertension in Connective Tissue Disease

verfasst von: Professor Dr. Ekkehard Grünig, MD

Erschienen in: Drugs | Ausgabe 8/2012

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Abstract

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.
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Metadaten
Titel
Treatment of Pulmonary Arterial Hypertension in Connective Tissue Disease
verfasst von
Professor Dr. Ekkehard Grünig, MD
Publikationsdatum
01.05.2012
Verlag
Springer International Publishing
Erschienen in
Drugs / Ausgabe 8/2012
Print ISSN: 0012-6667
Elektronische ISSN: 1179-1950
DOI
https://doi.org/10.2165/11633390-000000000-00000

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