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...
Saved in:
| Main Author: | |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
2012
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| In: |
Drugs
Year: 2012, Volume: 72, Issue: 8, Pages: 1039-1056 |
| ISSN: | 1179-1950 |
| DOI: | 10.2165/11633390-000000000-00000 |
| Online Access: | Verlag, Volltext: http://dx.doi.org/10.2165/11633390-000000000-00000 Verlag, Volltext: https://link.springer.com/article/10.2165/11633390-000000000-00000 
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| Author Notes: | Ekkehard Grünig |
MARC
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| 520 | |a 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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