Prognostic significance of computed tomography criteria for pulmonary veno-occlusive disease in systemic sclerosis-pulmonary arterial hypertension

Abstract

Objectives. SSc-pulmonary arterial hypertension (SSc-PAH) is associated with worse response to therapy and survival when compared with idiopathic PAH. It is suggested that the vasculopathy in SSc may involve postcapillary pulmonary venules resulting in pulmonary veno-occlusive disease (PVOD). This may underlie the lower gas transfer and worse outcome on therapy. We sought to test whether CT signs of PVOD (CTS-PVOD) were frequent in SSc-PAH and whether they were associated with pulmonary oedema on therapy and worse survival. Methods. CT thorax of 66 SSc patients with precapillary pulmonary hypertension (PH) were blindly scored by two radiologists for CTS-PVOD (≤1 or≥2). Case note and radiograph review determined the presence of pulmonary oedema on therapy. Results. Fifty-nine patients (89%) had ≤1 CTS-PVOD and only 7 (11%) had ≥2 CTS-PVOD. Pulmonary oedema on therapy was relatively common in those with ≥2 CTS-PVOD. On univariate analysis ≥2 CTS-PVOD were associated with a trend towards worse survival. Conclusion. CTS-PVOD were less frequent in this SSc-PAH cohort than in previous reports but the presence of at least two of these signs is associated with pulmonary oedema on therapy and a trend towards worse survival on univariate analysis.