Abstract
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.
- © 2011 ERS
