{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T05:57:09Z","timestamp":1790834229443,"version":"4.1.0"},"reference-count":27,"publisher":"European Respiratory Society (ERS)","issue":"2","funder":[{"DOI":"10.13039\/100005646","name":"Actelion Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005646","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2017,5,10]]},"published-print":{"date-parts":[[2017,8]]},"abstract":"<jats:p>Patients 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.<\/jats:p>\n                  <jats:p>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.<\/jats:p>\n                  <jats:p>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\u20130.85). 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.<\/jats:p>\n                  <jats:p>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.<\/jats:p>","DOI":"10.1183\/13993003.02493-2016","type":"journal-article","created":{"date-parts":[[2017,9,18]],"date-time":"2017-09-18T05:32:55Z","timestamp":1505712775000},"page":"1602493","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":129,"title":["Selexipag for the treatment of connective tissue disease-associated pulmonary arterial 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European Public Assessment Report: Uptravi (selexipag) 2016. www.ema.europa.eu\/docs\/en_GB\/document_library\/EPAR_-_Public_assessment_report\/human\/003774\/WC500207175.pdf Date last accessed: March 15, 2017. 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