PT  - JOURNAL ARTICLE
AU  - Hervier, Baptiste
AU  - Meyer, Alain
AU  - Dieval, Céline
AU  - Uzunhan, Yurdagul
AU  - Devilliers, Hervé
AU  - Launay, David
AU  - Canuet, Matthieu
AU  - Têtu, Laurent
AU  - Agard, Christian
AU  - Sibilia, Jean
AU  - Hamidou, Mohamed
AU  - Amoura, Zahir
AU  - Nunes, Hilario
AU  - Benveniste, Olivier
AU  - Grenier, Philippe
AU  - Montani, David
AU  - Hachulla, Eric
TI  - Pulmonary hypertension in antisynthetase syndrome: prevalence, etiology and survival
AID  - 10.1183/09031936.00156312
DP  - 2013 Jan 01
TA  - European Respiratory Journal
PG  - erj01563-2012
4099  - https://publications.ersnet.org//content/early/2013/01/23/09031936.00156312.short
4100  - https://publications.ersnet.org//content/early/2013/01/23/09031936.00156312.full
AB  - 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.