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10.1183/13993003.02493-2016

http://scihub22266oqcxt.onion/10.1183/13993003.02493-2016
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C5593379!5593379 !28818881
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suck abstract from ncbi


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pmid28818881
      Eur+Respir+J 2017 ; 50 (2 ): ä
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  • Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension #MMPMID28818881
  • Gaine S ; Chin K ; Coghlan G ; Channick R ; Di Scala L ; Galiè N ; Ghofrani HA ; Lang IM ; McLaughlin V ; Preiss R ; Rubin LJ ; Simonneau G ; Sitbon O ; Tapson VF ; Hoeper MM
  • Eur Respir J 2017[Aug]; 50 (2 ): ä PMID28818881 show ga
  • 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.
  • |*Acetamides/administration & dosage/adverse effects [MESH]
  • |*Hypertension, Pulmonary/diagnosis/drug therapy/etiology/mortality [MESH]
  • |*Pyrazines/administration & dosage/adverse effects [MESH]
  • |Adult [MESH]
  • |Antihypertensive Agents/administration & dosage/adverse effects [MESH]
  • |Disease Progression [MESH]
  • |Double-Blind Method [MESH]
  • |Female [MESH]
  • |Humans [MESH]
  • |Lupus Erythematosus, Systemic/*complications [MESH]
  • |Male [MESH]
  • |Middle Aged [MESH]
  • |Outcome Assessment, Health Care [MESH]
  • |Risk Assessment [MESH]
  • |Scleroderma, Systemic/*complications [MESH]


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