Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil
Identifieur interne : 003821 ( Main/Exploration ); précédent : 003820; suivant : 003822Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil
Auteurs : David Launay [France] ; Olivier Sitbon [France] ; Jérôme Le Pavec [France] ; Laurent Savale [France] ; Colas Tchérakian [France] ; Azzedine Yaïci [France] ; Lara Achouh [France] ; Florence Parent [France] ; Xavier Jais [France] ; Gérald Simonneau [France] ; Marc Humbert [France]Source :
- Rheumatology [ 1462-0324 ] ; 2010-03.
Abstract
Objective. Data on long-term efficacy of bosentan, an oral dual ET receptor antagonist, in SSc-associated pulmonary arterial hypertension (SSc-PAH) are lacking. We aimed to describe the long-term outcome of SSc-PAH treated with first-line monotherapy bosentan followed or not by the addition of prostanoids or sildenafil. Methods. A prospective analysis of 49 consecutive SSc-PAH patients treated with first-line bosentan was performed. New York Heart Association (NYHA) functional class, 6-min walk distance (6MWD) and haemodynamics were assessed at baseline and after 4 and 12 months. Results. At 4 months, significant improvements in NYHA functional class and haemodynamics were observed with stabilization at 1 year. There was no significant improvement in 6MWD. Overall survival estimates were 80, 56 and 51% at 1, 2 and 3 years, respectively, and were significantly worse than those in a cohort of patients with idiopathic PAH (92, 89 and 79% at 1, 2 and 3 years, respectively; P < 0.0001). Twenty-three patients (47%) died after a mean follow-up of 23 (18) months. In multivariate analysis, baseline and 4-month NYHA functional class and 4-month cardiac index were independent factors associated with overall survival. Conclusions. In our cohort of consecutive SSc-PAH patients treated with first-line bosentan, improvement in NYHA functional class and haemodynamics was significant after 4 months of treatment and stabilized afterwards. One-year overall survival rate was higher than previously reported in historical series. However, long-term prognosis remains poor. Our study underlines the importance of haemodynamic evaluation 4 months after the start of treatment to provide strong parameters associated with survival-like cardiac index.
Url:
DOI: 10.1093/rheumatology/kep398
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream Istex, to step Corpus: 002D62
- to stream Istex, to step Curation: 002D25
- to stream Istex, to step Checkpoint: 000926
- to stream Main, to step Merge: 003899
- to stream Main, to step Curation: 003821
Le document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title>Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil</title>
<author><name sortKey="Launay, David" sort="Launay, David" uniqKey="Launay D" first="David" last="Launay">David Launay</name>
</author>
<author><name sortKey="Sitbon, Olivier" sort="Sitbon, Olivier" uniqKey="Sitbon O" first="Olivier" last="Sitbon">Olivier Sitbon</name>
</author>
<author><name sortKey="Le Pavec, Jerome" sort="Le Pavec, Jerome" uniqKey="Le Pavec J" first="Jérôme" last="Le Pavec">Jérôme Le Pavec</name>
</author>
<author><name sortKey="Savale, Laurent" sort="Savale, Laurent" uniqKey="Savale L" first="Laurent" last="Savale">Laurent Savale</name>
</author>
<author><name sortKey="Tcherakian, Colas" sort="Tcherakian, Colas" uniqKey="Tcherakian C" first="Colas" last="Tchérakian">Colas Tchérakian</name>
</author>
<author><name sortKey="Yaici, Azzedine" sort="Yaici, Azzedine" uniqKey="Yaici A" first="Azzedine" last="Yaïci">Azzedine Yaïci</name>
</author>
<author><name sortKey="Achouh, Lara" sort="Achouh, Lara" uniqKey="Achouh L" first="Lara" last="Achouh">Lara Achouh</name>
</author>
<author><name sortKey="Parent, Florence" sort="Parent, Florence" uniqKey="Parent F" first="Florence" last="Parent">Florence Parent</name>
</author>
<author><name sortKey="Jais, Xavier" sort="Jais, Xavier" uniqKey="Jais X" first="Xavier" last="Jais">Xavier Jais</name>
</author>
<author><name sortKey="Simonneau, Gerald" sort="Simonneau, Gerald" uniqKey="Simonneau G" first="Gérald" last="Simonneau">Gérald Simonneau</name>
</author>
<author><name sortKey="Humbert, Marc" sort="Humbert, Marc" uniqKey="Humbert M" first="Marc" last="Humbert">Marc Humbert</name>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">ISTEX</idno>
<idno type="RBID">ISTEX:C051F5771630C7B69C5809E8F2EAD7EC56313FDD</idno>
<date when="2009" year="2009">2009</date>
<idno type="doi">10.1093/rheumatology/kep398</idno>
<idno type="url">https://api.istex.fr/ark:/67375/HXZ-VSPVJVWT-S/fulltext.pdf</idno>
<idno type="wicri:Area/Istex/Corpus">002D62</idno>
<idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">002D62</idno>
<idno type="wicri:Area/Istex/Curation">002D25</idno>
<idno type="wicri:Area/Istex/Checkpoint">000926</idno>
<idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Checkpoint">000926</idno>
<idno type="wicri:doubleKey">1462-0324:2009:Launay D:long:term:outcome</idno>
<idno type="wicri:Area/Main/Merge">003899</idno>
<idno type="wicri:Area/Main/Curation">003821</idno>
<idno type="wicri:Area/Main/Exploration">003821</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title level="a">Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil</title>
<author><name sortKey="Launay, David" sort="Launay, David" uniqKey="Launay D" first="David" last="Launay">David Launay</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Sitbon, Olivier" sort="Sitbon, Olivier" uniqKey="Sitbon O" first="Olivier" last="Sitbon">Olivier Sitbon</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
<affiliation wicri:level="1"><country wicri:rule="url">France</country>
</affiliation>
</author>
<author><name sortKey="Le Pavec, Jerome" sort="Le Pavec, Jerome" uniqKey="Le Pavec J" first="Jérôme" last="Le Pavec">Jérôme Le Pavec</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Savale, Laurent" sort="Savale, Laurent" uniqKey="Savale L" first="Laurent" last="Savale">Laurent Savale</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Tcherakian, Colas" sort="Tcherakian, Colas" uniqKey="Tcherakian C" first="Colas" last="Tchérakian">Colas Tchérakian</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Yaici, Azzedine" sort="Yaici, Azzedine" uniqKey="Yaici A" first="Azzedine" last="Yaïci">Azzedine Yaïci</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Achouh, Lara" sort="Achouh, Lara" uniqKey="Achouh L" first="Lara" last="Achouh">Lara Achouh</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Parent, Florence" sort="Parent, Florence" uniqKey="Parent F" first="Florence" last="Parent">Florence Parent</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Jais, Xavier" sort="Jais, Xavier" uniqKey="Jais X" first="Xavier" last="Jais">Xavier Jais</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Simonneau, Gerald" sort="Simonneau, Gerald" uniqKey="Simonneau G" first="Gérald" last="Simonneau">Gérald Simonneau</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
<author><name sortKey="Humbert, Marc" sort="Humbert, Marc" uniqKey="Humbert M" first="Marc" last="Humbert">Marc Humbert</name>
<affiliation wicri:level="4"><country xml:lang="fr">France</country>
<wicri:regionArea>Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre, AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart, INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson and Centre de Référence de la Sclérodermie Systémique, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille</wicri:regionArea>
<placeName><region type="region">Hauts-de-France</region>
<region type="old region">Nord-Pas-de-Calais</region>
<settlement type="city">Lille</settlement>
</placeName>
<orgName type="university">Université Paris-Sud</orgName>
</affiliation>
</author>
</analytic>
<monogr></monogr>
<series><title level="j">Rheumatology</title>
<idno type="ISSN">1462-0324</idno>
<idno type="eISSN">1462-0332</idno>
<imprint><publisher>Oxford University Press</publisher>
<date type="published" when="2010-03">2010-03</date>
<biblScope unit="volume">49</biblScope>
<biblScope unit="issue">3</biblScope>
<biblScope unit="page" from="490">490</biblScope>
<biblScope unit="page" to="500">500</biblScope>
</imprint>
<idno type="ISSN">1462-0324</idno>
</series>
</biblStruct>
</sourceDesc>
<seriesStmt><idno type="ISSN">1462-0324</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract">Objective. Data on long-term efficacy of bosentan, an oral dual ET receptor antagonist, in SSc-associated pulmonary arterial hypertension (SSc-PAH) are lacking. We aimed to describe the long-term outcome of SSc-PAH treated with first-line monotherapy bosentan followed or not by the addition of prostanoids or sildenafil. Methods. A prospective analysis of 49 consecutive SSc-PAH patients treated with first-line bosentan was performed. New York Heart Association (NYHA) functional class, 6-min walk distance (6MWD) and haemodynamics were assessed at baseline and after 4 and 12 months. Results. At 4 months, significant improvements in NYHA functional class and haemodynamics were observed with stabilization at 1 year. There was no significant improvement in 6MWD. Overall survival estimates were 80, 56 and 51% at 1, 2 and 3 years, respectively, and were significantly worse than those in a cohort of patients with idiopathic PAH (92, 89 and 79% at 1, 2 and 3 years, respectively; P < 0.0001). Twenty-three patients (47%) died after a mean follow-up of 23 (18) months. In multivariate analysis, baseline and 4-month NYHA functional class and 4-month cardiac index were independent factors associated with overall survival. Conclusions. In our cohort of consecutive SSc-PAH patients treated with first-line bosentan, improvement in NYHA functional class and haemodynamics was significant after 4 months of treatment and stabilized afterwards. One-year overall survival rate was higher than previously reported in historical series. However, long-term prognosis remains poor. Our study underlines the importance of haemodynamic evaluation 4 months after the start of treatment to provide strong parameters associated with survival-like cardiac index.</div>
</front>
</TEI>
<affiliations><list><country><li>France</li>
</country>
<region><li>Hauts-de-France</li>
<li>Nord-Pas-de-Calais</li>
</region>
<settlement><li>Lille</li>
</settlement>
<orgName><li>Université Paris-Sud</li>
</orgName>
</list>
<tree><country name="France"><region name="Hauts-de-France"><name sortKey="Launay, David" sort="Launay, David" uniqKey="Launay D" first="David" last="Launay">David Launay</name>
</region>
<name sortKey="Achouh, Lara" sort="Achouh, Lara" uniqKey="Achouh L" first="Lara" last="Achouh">Lara Achouh</name>
<name sortKey="Humbert, Marc" sort="Humbert, Marc" uniqKey="Humbert M" first="Marc" last="Humbert">Marc Humbert</name>
<name sortKey="Jais, Xavier" sort="Jais, Xavier" uniqKey="Jais X" first="Xavier" last="Jais">Xavier Jais</name>
<name sortKey="Le Pavec, Jerome" sort="Le Pavec, Jerome" uniqKey="Le Pavec J" first="Jérôme" last="Le Pavec">Jérôme Le Pavec</name>
<name sortKey="Parent, Florence" sort="Parent, Florence" uniqKey="Parent F" first="Florence" last="Parent">Florence Parent</name>
<name sortKey="Savale, Laurent" sort="Savale, Laurent" uniqKey="Savale L" first="Laurent" last="Savale">Laurent Savale</name>
<name sortKey="Simonneau, Gerald" sort="Simonneau, Gerald" uniqKey="Simonneau G" first="Gérald" last="Simonneau">Gérald Simonneau</name>
<name sortKey="Sitbon, Olivier" sort="Sitbon, Olivier" uniqKey="Sitbon O" first="Olivier" last="Sitbon">Olivier Sitbon</name>
<name sortKey="Sitbon, Olivier" sort="Sitbon, Olivier" uniqKey="Sitbon O" first="Olivier" last="Sitbon">Olivier Sitbon</name>
<name sortKey="Tcherakian, Colas" sort="Tcherakian, Colas" uniqKey="Tcherakian C" first="Colas" last="Tchérakian">Colas Tchérakian</name>
<name sortKey="Yaici, Azzedine" sort="Yaici, Azzedine" uniqKey="Yaici A" first="Azzedine" last="Yaïci">Azzedine Yaïci</name>
</country>
</tree>
</affiliations>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Lorraine/explor/InforLorV4/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 003821 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 003821 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Lorraine |area= InforLorV4 |flux= Main |étape= Exploration |type= RBID |clé= ISTEX:C051F5771630C7B69C5809E8F2EAD7EC56313FDD |texte= Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil }}
This area was generated with Dilib version V0.6.33. |