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

Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein Ardeschir Ghofrani, Irene M. Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J. Rubin, Gérald Simonneau, Olivier Sitbon, Victor F. Tapson, Marius M. Hoeper

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90 Scopus citations


Patients with connective tissue disease-associated pulmonary arterial hypertension (PAHCTD) 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 prostacyclinrelated 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.

Original languageEnglish (US)
JournalEuropean Respiratory Journal
Issue number2
StatePublished - Aug 1 2017

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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