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Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.
Gaine, Sean; Chin, Kelly; Coghlan, Gerry; Channick, Richard; Di Scala, Lilla; Galiè, Nazzareno; Ghofrani, Hossein-Ardeschir; Lang, Irene M; McLaughlin, Vallerie; Preiss, Ralph; Rubin, Lewis J; Simonneau, Gérald; Sitbon, Olivier; Tapson, Victor F; Hoeper, Marius M.
Afiliação
  • Gaine S; National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland sgaine@mater.ie.
  • Chin K; University of Texas Southwestern, Medical Centre, Dallas, TX, USA.
  • Coghlan G; Cardiology Dept, Royal Free Hospital, London, UK.
  • Channick R; Pulmonary and Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
  • Di Scala L; Actelion Pharmaceuticals Ltd, Allschwil, Switzerland.
  • Galiè N; Dept of Experimental, Diagnostic and Specialty Medicine (DIMES), Bologna University Hospital, Bologna, Italy.
  • Ghofrani HA; University of Giessen and Marburg Lung Center, Giessen, Germany.
  • Lang IM; German Center of Lung Research (DZL), Giessen, Germany.
  • McLaughlin V; Dept of Medicine, Imperial College London, London, UK.
  • Preiss R; Dept of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Allgemeines Krankenhaus, Vienna, Austria.
  • Rubin LJ; Dept of Internal Medicine, University of Michigan Health System, Ann Arbor, MI, USA.
  • Simonneau G; Actelion Pharmaceuticals Ltd, Allschwil, Switzerland.
  • Sitbon O; Dept of Medicine, University of California, San Diego, CA, USA.
  • Tapson VF; Assistance Publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
  • Hoeper MM; Université Paris-Sud, Laboratoire d'Excellence en Recherche sur le Médicament et Innovation Thérapeutique, Le Kremlin-Bicêtre, France.
Eur Respir J ; 50(2)2017 08.
Article em En | MEDLINE | ID: mdl-28818881
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models.Compared with the overall GRIPHON population, the CTD subgroup was slightly older with a greater proportion of females and shorter time since diagnosis. Patients with PAH-SSc appeared to be more impaired at baseline, with a more progressive disease course. The converse was observed for PAH-SLE. Selexipag reduced the risk of composite morbidity/mortality events in patients with PAH-CTD by 41% (HR 0.59; 95% CI 0.41-0.85). Treatment effect was consistent irrespective of baseline PAH therapy or CTD subtype (interaction p=0.87 and 0.89, respectively). Adverse events were predominately prostacyclin-related and known for selexipag treatment.GRIPHON has allowed the comprehensive characterisation of patients with PAH-CTD. Selexipag delayed progression of PAH and was well-tolerated among PAH-CTD patients, including those with PAH-SSc and PAH-SLE.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Tipo de estudo: Clinical_trials / Diagnostic_studies / Etiology_studies / Prognostic_studies / Risk_factors_studies Limite: Adult / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2017 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Tipo de estudo: Clinical_trials / Diagnostic_studies / Etiology_studies / Prognostic_studies / Risk_factors_studies Limite: Adult / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2017 Tipo de documento: Article