Journalartikel
Autorenliste: Gaine, Sean; Chin, Kelly; Coghlan, Gerry; Channick, Richard; Di Scala, Lilla; Galie, Nazzareno; Ghofrani, Hossein-Ardeschir; Lang, Irene M.; McLaughlin, Vallerie; Preiss, Ralph; Rubin, Lewis J.; Simonneau, Gerald; Sitbon, Olivier; Tapson, Victor F.; Hoeper, Marius M.
Jahr der Veröffentlichung: 2017
Zeitschrift: European Respiratory Journal
Bandnummer: 50
Heftnummer: 2
ISSN: 0903-1936
eISSN: 1399-3003
Open Access Status: Hybrid
DOI Link: https://doi.org/10.1183/13993003.02493-2016
Verlag: European Respiratory Society
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.
Abstract:
Zitierstile
Harvard-Zitierstil: 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-Zitierstil: 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
Schlagwörter
5 INHIBITOR THERAPY; 6-minute walk distance; ENDOTHELIN RECEPTOR ANTAGONIST; MONOTHERAPY; ORAL TREPROSTINIL; RANDOMIZED CONTROLLED-TRIAL; SCLEROSIS; SYSTEMIC-LUPUS-ERYTHEMATOSUS
