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Pulmonary hypertension in antisynthetase syndrome: prevalence, etiology and survival

Baptiste Hervier, Alain Meyer, Céline Dieval, Yurdagul Uzunhan, Hervé Devilliers, David Launay, Matthieu Canuet, Laurent Têtu, Christian Agard, Jean Sibilia, Mohamed Hamidou, Zahir Amoura, Hilario Nunes, Olivier Benveniste, Philippe Grenier, David Montani, Eric Hachulla
European Respiratory Journal 2013; DOI: 10.1183/09031936.00156312
Baptiste Hervier
*Hôpital Pitié-Salpêtrière, APHP, Paris, France & University of Paris VI Pierre & Marie Curie; Internal Medicine dept 2 & INSERM UMRS-945, French Reference Center for Lupus
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  • For correspondence: bhervier@yahoo.fr
Alain Meyer
#Strasbourg university hospital, Strasbourg France, Rheumatology dept, French reference center for Systemic rare diseases
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Céline Dieval
¶Internal Medicine & Infectious diseases dept, St-André Hospital, University of Bordeaux, Bordeaux, France
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Yurdagul Uzunhan
+University of Paris 13, Sorbonne Paris Cité, EA 2363; AP-HP, Dept of Pneumology, Avicenne hospital, Bobigny, France
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Hervé Devilliers
§Internal Medicine & Systemic disease dept, University hospital of Dijon, Dijon, France
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David Launay
fInternal Medicine dept, French National Center for Rare Systemic Auto-Immune Diseases (Scleroderma), Claude Huriez Hospital, Lille 2 University, Lille, France
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Matthieu Canuet
#Strasbourg university hospital, Strasbourg France, Rheumatology dept, French reference center for Systemic rare diseases
ffStrasbourg university hospital, Strasbourg France, Pneumology dept
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Laurent Têtu
**Pneumology dept, Larrey Hospital, Paul Sabatier university, Toulouse, France
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Christian Agard
##Internal Medicine dept, Hôtel Dieu, Nantes university, Nantes, France
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Jean Sibilia
#Strasbourg university hospital, Strasbourg France, Rheumatology dept, French reference center for Systemic rare diseases
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Mohamed Hamidou
##Internal Medicine dept, Hôtel Dieu, Nantes university, Nantes, France
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Zahir Amoura
*Hôpital Pitié-Salpêtrière, APHP, Paris, France & University of Paris VI Pierre & Marie Curie; Internal Medicine dept 2 & INSERM UMRS-945, French Reference Center for Lupus
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Hilario Nunes
+University of Paris 13, Sorbonne Paris Cité, EA 2363; AP-HP, Dept of Pneumology, Avicenne hospital, Bobigny, France
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Olivier Benveniste
*Hôpital Pitié-Salpêtrière, APHP, Paris, France & University of Paris VI Pierre & Marie Curie; Internal Medicine dept 2 & INSERM UMRS-945, French Reference Center for Lupus
++Hôpital Pitié-Salpêtrière, APHP, Paris, France & University of Paris VI Pierre & Marie Curie; Internal Medicine dept 1, French Reference Center for Neuromuscular Disorders
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Philippe Grenier
*Hôpital Pitié-Salpêtrière, APHP, Paris, France & University of Paris VI Pierre & Marie Curie; Internal Medicine dept 2 & INSERM UMRS-945, French Reference Center for Lupus
§§Hôpital Pitié-Salpêtrière, APHP, Paris, France & University of Paris VI Pierre & Marie Curie; Radiology dept
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David Montani
¶¶Université Paris-Sud, Centre de Référence de l’Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, AP-HP, DHU Thorax Innovation, INSERM U999, Hôpital de Bicêtre, Le Kremlin-Bicêtre, F-94270, France
***David Montani and Eric Hachulla contributed equally to the work
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Eric Hachulla
fInternal Medicine dept, French National Center for Rare Systemic Auto-Immune Diseases (Scleroderma), Claude Huriez Hospital, Lille 2 University, Lille, France
***David Montani and Eric Hachulla contributed equally to the work
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Abstract

Antisynthetase syndrome (ASS) is characterized by the association of interstitial lung disease (ILD) and myositis with different anti-tRNA-synthetase antibodies. The occurrence, etiology and prognosis of pulmonary hypertension (PH) have not yet been evaluated.

Among 203 consecutive patients, echocardiography (TTE) and right heart catheterization (RHC) results were retrospectively analysed in light of clinico-biological, morphological and functional parameters. Definitions of PH were based on the ESC/ERS 2009 guidelines, severe PH being defined by a mean pulmonary arterial pressure (mPAP) >35 mmHg.

PH was suspected by TTE in 47 (23.2%) cases, corresponding to PH “possible” (n=27, 13.3%) or “likely” (n=20, 9.9%). RHC was performed in 21 patients, excluding PH in 5 and confirming pre-capillary PH in 16 (7.9%). Although related to ILD in all cases, pre-capillary PH was severe in 13 (81,3%) patients (mean mPAP: 46±9 mmHg), frequently associated with low cardiac index (mean 2.3±0.8 l·min−1·m−2) and high FVC/DL,CO ratio (2.5±0.6). PH was clearly associated with a lower survival (p<0.001), with a 3-year survival rate of 58%.

The occurrence of PH in ASS is significant and dramatically worsens the prognosis. Although systematically associated with ILD, PH was usually severe, suggesting a specific pulmonary vascular involvement.

  • Anti-tRNA-synthetase antibody
  • interstitial lung disease
  • myositis
  • pulmonary hypertension
  • ERS
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European Respiratory Journal: 62 (3)
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Pulmonary hypertension in antisynthetase syndrome: prevalence, etiology and survival
Baptiste Hervier, Alain Meyer, Céline Dieval, Yurdagul Uzunhan, Hervé Devilliers, David Launay, Matthieu Canuet, Laurent Têtu, Christian Agard, Jean Sibilia, Mohamed Hamidou, Zahir Amoura, Hilario Nunes, Olivier Benveniste, Philippe Grenier, David Montani, Eric Hachulla
European Respiratory Journal Jan 2013, erj01563-2012; DOI: 10.1183/09031936.00156312

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Pulmonary hypertension in antisynthetase syndrome: prevalence, etiology and survival
Baptiste Hervier, Alain Meyer, Céline Dieval, Yurdagul Uzunhan, Hervé Devilliers, David Launay, Matthieu Canuet, Laurent Têtu, Christian Agard, Jean Sibilia, Mohamed Hamidou, Zahir Amoura, Hilario Nunes, Olivier Benveniste, Philippe Grenier, David Montani, Eric Hachulla
European Respiratory Journal Jan 2013, erj01563-2012; DOI: 10.1183/09031936.00156312
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