TY - JOUR
T1 - Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension
AU - Gaine, Sean
AU - Chin, Kelly
AU - Coghlan, Gerry
AU - Channick, Richard
AU - Di Scala, Lilla
AU - Galiè, Nazzareno
AU - Ghofrani, Hossein Ardeschir
AU - Lang, Irene M.
AU - McLaughlin, Vallerie
AU - Preiss, Ralph
AU - Rubin, Lewis J.
AU - Simonneau, Gérald
AU - Sitbon, Olivier
AU - Tapson, Victor F.
AU - Hoeper, Marius M.
N1 - Funding Information:
Support statement: This study was supported by Actelion Pharmaceuticals. Funding information for this article has been deposited with the Crossref Funder Registry.
Funding Information:
Medical writing assistance was provided by Anoushka Thomas (nspm ltd, Meggen, Switzerland) and funded by Actelion Pharmaceuticals Ltd (Allschwil, Switzerland).
Publisher Copyright:
© ERS 2017.
PY - 2017/8/1
Y1 - 2017/8/1
N2 - 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.
AB - 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.
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U2 - 10.1183/13993003.02493-2016
DO - 10.1183/13993003.02493-2016
M3 - Article
C2 - 28818881
AN - SCOPUS:85027881389
SN - 0903-1936
VL - 50
JO - European Respiratory Journal
JF - European Respiratory Journal
IS - 2
ER -