Gaine, SeanChin, KellyCoghlan, GerryChannick, RichardDi Scala, LillaGaliè, NazzarenoGhofrani, Hossein-ArdeschirLang, Irene M.McLaughlin, ValleriePreiss, RalphRubin, Lewis J.Simonneau, GéraldSitbon, OlivierTapson, Victor F.Hoeper, Marius M.2017-12-052017Gaine, S., K. Chin, G. Coghlan, R. Channick, L. Di Scala, N. Galiè, H. Ghofrani, et al. 2017. “Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.” The European Respiratory Journal 50 (2): 1602493. doi:10.1183/13993003.02493-2016. http://dx.doi.org/10.1183/13993003.02493-2016.http://nrs.harvard.edu/urn-3:HUL.InstRepos:34491946Patients 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.en-USPulmonary Vascular DiseasesJournal Article2017-12-0510.1183/13993003.02493-2016