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Treatment of Pulmonary Arterial Hypertension in Connective Tissue Disease

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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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Acknowledgements

Medical writing assistance was provided by Raelene Simpson and Nicola Ryan of inScience Communications. This assistance was funded by GlaxoSmithKline, Germany.

Within the past 12 months, EG has received honoraria for lectures from Actelion, Bayer, Encysive Pharmaceuticals, GSK, Lilly, Miltenyi Biotec, Pfizer and Rotex Medica; and received funding for clinical trials from Actelion, Bayer, GSK, Encysive Pharmaceuticals, Lilly, Mundogen and Pfizer.

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  1. Centre of Pulmonary Hypertension, Thoraxclinic, University Hospital Heidelberg, Amalienstrasse 5, D-69126, Heidelberg, Germany

    Ekkehard Grünig MD

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Grünig, E. Treatment of Pulmonary Arterial Hypertension in Connective Tissue Disease. Drugs 72, 1039–1056 (2012). https://doi.org/10.2165/11633390-000000000-00000

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