title: Selexipag for the treatment of pulmonary arterial hypertension.
abstract: BACKGROUND
  Targeted pulmonary vasoactive substances are the cornerstone of treatment in pulmonary arterial hypertension (PAH). Approved drugs act on various receptors and molecules within the pulmonary arteries, mainly causing pulmonary vasodilation and potentially reversing remodeling with consequent improvement of right ventricular function. A key role is attributed to the prostacyclin pathway and especially the prostacyclin receptor (IP). Selexipag is a recently developed, non-prostanoid, oral IP receptor agonist for the treatment of PAH which has been approved in countries/regions including the USA and Europe.
  
  
  METHODS
  We review the discovery and development of drugs targeting IP receptors in PAH and describe preclinical and phase I studies of selexipag. Furthermore, we review important phase II and III selexipag studies and place them into the clinical context of previously approved prostanoids.
  
  
  CONCLUSIONS
  Oral selexipag offers a promising therapeutic option within the class of available drugs targeting IP receptors. However, its role as first-line therapy based on its efficacy/side-effect profile in current studies is questionable. Most likely, selexipag will be used in combination with other PAH-specific oral drugs. The potential of selexipag to replace or postpone the use of inhaled or parenteral prostanoids needs to be investigated in future trials.
authors.affiliation: a Department of Internal Medicine , University of Giessen and Marburg Lung Center (UGMLC) , Giessen , Germany, member of the German Center of Lung Research (DZL).|b Department of Pneumology , Kerckhoff Heart and Thoracic Center , Bad Nauheim , Germany.
authors.forename: Manuel Jonas
authors.initials: MJ
authors.lastname: Richter
authors.affiliation: a Department of Internal Medicine , University of Giessen and Marburg Lung Center (UGMLC) , Giessen , Germany, member of the German Center of Lung Research (DZL).
authors.forename: Henning
authors.initials: H
authors.lastname: Gall
authors.affiliation: a Department of Internal Medicine , University of Giessen and Marburg Lung Center (UGMLC) , Giessen , Germany, member of the German Center of Lung Research (DZL).|c Center for Pulmonary Hypertension , University Medical Center Hamburg-Eppendorf , Hamburg , Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Grimminger
authors.affiliation: a Department of Internal Medicine , University of Giessen and Marburg Lung Center (UGMLC) , Giessen , Germany, member of the German Center of Lung Research (DZL).
authors.forename: Friedrich
authors.initials: F
authors.lastname: Grimminger
authors.affiliation: a Department of Internal Medicine , University of Giessen and Marburg Lung Center (UGMLC) , Giessen , Germany, member of the German Center of Lung Research (DZL).|b Department of Pneumology , Kerckhoff Heart and Thoracic Center , Bad Nauheim , Germany.|d Department of Medicine , Imperial College London , London , UK.
authors.forename: Hossein-Ardeschir
authors.initials: HA
authors.lastname: Ghofrani
mesh_terms: D000081:Acetamides / Q000627:therapeutic use*; D000284:Administration, Oral; D000818:Animals; D017277:Drug Approval; D011464:Epoprostenol / Q000378:metabolism; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D011719:Pyrazines / Q000627:therapeutic use*
chemical_list: D000081:Acetamides; D011719:Pyrazines; C523468:selexipag; D011464:Epoprostenol
keywords: Prostacyclin receptor agonist; pulmonary arterial hypertension; selexipag; treatment