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

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 selexipa...

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Autores principales: Gaine, Sean, Chin, Kelly, Coghlan, Gerry, Channick, Richard, Di Scala, Lilla, Galiè, Nazzareno, Ghofrani, Hossein-Ardeschir, Lang, Irene M., McLaughlin, Vallerie, Preiss, Ralph, Rubin, Lewis J., Simonneau, Gérald, Sitbon, Olivier, Tapson, Victor F., Hoeper, Marius M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5593379/
https://www.ncbi.nlm.nih.gov/pubmed/28818881
http://dx.doi.org/10.1183/13993003.02493-2016
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author Gaine, Sean
Chin, Kelly
Coghlan, Gerry
Channick, Richard
Di Scala, Lilla
Galiè, Nazzareno
Ghofrani, Hossein-Ardeschir
Lang, Irene M.
McLaughlin, Vallerie
Preiss, Ralph
Rubin, Lewis J.
Simonneau, Gérald
Sitbon, Olivier
Tapson, Victor F.
Hoeper, Marius M.
author_facet Gaine, Sean
Chin, Kelly
Coghlan, Gerry
Channick, Richard
Di Scala, Lilla
Galiè, Nazzareno
Ghofrani, Hossein-Ardeschir
Lang, Irene M.
McLaughlin, Vallerie
Preiss, Ralph
Rubin, Lewis J.
Simonneau, Gérald
Sitbon, Olivier
Tapson, Victor F.
Hoeper, Marius M.
author_sort Gaine, Sean
collection PubMed
description 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. 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 prostacyclin-related 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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spelling pubmed-55933792017-09-18 Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension Gaine, Sean Chin, Kelly Coghlan, Gerry Channick, Richard Di Scala, Lilla Galiè, Nazzareno Ghofrani, Hossein-Ardeschir Lang, Irene M. McLaughlin, Vallerie Preiss, Ralph Rubin, Lewis J. Simonneau, Gérald Sitbon, Olivier Tapson, Victor F. Hoeper, Marius M. Eur Respir J Original Articles 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. 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 prostacyclin-related 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. European Respiratory Society 2017-08-17 /pmc/articles/PMC5593379/ /pubmed/28818881 http://dx.doi.org/10.1183/13993003.02493-2016 Text en Copyright ©ERS 2017. http://creativecommons.org/licenses/by-nc/4.0/ This ERJ Open article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
spellingShingle Original Articles
Gaine, Sean
Chin, Kelly
Coghlan, Gerry
Channick, Richard
Di Scala, Lilla
Galiè, Nazzareno
Ghofrani, Hossein-Ardeschir
Lang, Irene M.
McLaughlin, Vallerie
Preiss, Ralph
Rubin, Lewis J.
Simonneau, Gérald
Sitbon, Olivier
Tapson, Victor F.
Hoeper, Marius M.
Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension
title Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension
title_full Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension
title_fullStr Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension
title_full_unstemmed Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension
title_short Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension
title_sort selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5593379/
https://www.ncbi.nlm.nih.gov/pubmed/28818881
http://dx.doi.org/10.1183/13993003.02493-2016
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