TY - JOUR T1 - Pulmonary hypertension in antisynthetase syndrome: prevalence, etiology and survival JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/09031936.00156312 SP - erj01563-2012 AU - Baptiste Hervier AU - Alain Meyer AU - Céline Dieval AU - Yurdagul Uzunhan AU - Hervé Devilliers AU - David Launay AU - Matthieu Canuet AU - Laurent Têtu AU - Christian Agard AU - Jean Sibilia AU - Mohamed Hamidou AU - Zahir Amoura AU - Hilario Nunes AU - Olivier Benveniste AU - Philippe Grenier AU - David Montani AU - Eric Hachulla Y1 - 2013/01/01 UR - http://erj.ersjournals.com/content/early/2013/01/23/09031936.00156312.abstract N2 - 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. ER -