Journal article

Publication year: 2017

Journal: European Respiratory Journal

Volume number: 50

Issue number: 2

ISSN: 0903-1936

eISSN: 1399-3003

Open access status: Hybrid

DOI Link: https://doi.org/10.1183/13993003.02493-2016

Publisher: European Respiratory Society

Abstract: 

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.

Harvard Citation style: Gaine, S., Chin, K., Coghlan, G., Channick, R., Di Scala, L., Galie, N., et al. (2017) Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension, European Respiratory Journal, 50(2), Article 1602493. https://doi.org/10.1183/13993003.02493-2016

APA Citation style: Gaine, S., Chin, K., Coghlan, G., Channick, R., Di Scala, L., Galie, N., Ghofrani, H., Lang, I., McLaughlin, V., Preiss, R., Rubin, L., Simonneau, G., Sitbon, O., Tapson, V., & Hoeper, M. (2017). Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension. European Respiratory Journal. 50(2), Article 1602493. https://doi.org/10.1183/13993003.02493-2016