Details

Autor(en) / Beteiligte

• 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

Titel

Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension

Ist Teil von

• The European respiratory journal, 2017-08, Vol.50 (2), p.1602493-1602493

Ort / Verlag

England: European Respiratory Society Journals Ltd

Erscheinungsjahr

2017

Link zum Volltext

Quelle

MEDLINE

Beschreibungen/Notizen

• 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.