title: Treatment of pulmonary arterial hypertension in connective tissue disease.
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 represents 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.
authors.affiliation: Centre for Pulmonary Hypertension, Thoraxclinic, University Hospital Heidelberg, Heidelberg, Germany. ekkehard.gruenig@thoraxklinik-heidelberg.de
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grünig
mesh_terms: D003240:Connective Tissue Diseases / Q000150:complications / Q000188:drug therapy*; D065627:Familial Primary Pulmonary Hypertension; D006333:Heart Failure / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy*; D016032:Randomized Controlled Trials as Topic