RT Journal Article
SR Electronic
T1 Frequency and impact on prognosis of signs of pulmonary veno-occlusive disease on high resolution computed tomography in patients with scleroderma associated pulmonary arterial hypertension
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p1525
VO 38
IS Suppl 55
A1 Sven Günther
A1 Sophie Maitre
A1 Alice Berezne
A1 Xavier Jaïs
A1 Andrei Seferian
A1 Olivier Sitbon
A1 Gerald Simonneau
A1 Luc Mouthon
A1 Marc Humbert
A1 David Montani
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p1525.abstract
AB Introduction: Pulmonary veno-occlusive disease (PVOD) is an uncommon form of pulmonary arterial hypertension (PAH) characterised by a progressive obstruction of small pulmonary veins. PVOD has been frequently reported in patients with scleroderma related PAH (SSc-PAH). High resolution chest computed tomography (HRCT) is a non-invasive diagnostic tool used to screen for PVOD. However, no data are available in SSc-PAH patients.Aims: To evaluate the frequency and the impact on prognosis of signs of PVOD on HRCT in SSc-PAH.Methods: We reviewed HRCT data in 34 consecutive SSc-PAH patients and 30 systemic sclerosis (SSc) patients.Results: Lymph nodes enlargement (57.7% vs 3.6%), centrilobular ground-glass opacities (46.2% vs 10.7%) and septal lines (73.1% vs 7.1%) were significantly more frequent in SSc-PAH patients as compared to SSc patients (all P<0.005). Indeed, 61.5% of SSc-PAH had ≥2 radiological signs of PVOD on HRCT. 53.8% of SSc-PAH patients had evidence of pericardial effusion (P<0.001). Cardiomegaly and pulmonary artery enlargement were significantly more frequently observed in SSC-PAH patients (P<0.001). Pleural effusion was observed in one patient (3.8%) in the group SSc-PAH, whereas no SSc patient had a pleural effusion. Survival in SSc-PAH patients with ≥2 radiological signs of PVOD was significant lower compared to those ≤1 radiological sign of PVOD (P<0.05).Conclusion: Signs of PVOD are frequent on HRCT in patients with SSc-PAH compared to SSc patients without PAH. These signs allow clinicians to detect PVOD in SSC-PAH patients. Survival in affected patients is poor.