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

Rheumatology (Oxford). 2017 Dec 1;56(12):2197-2203. doi: 10.1093/rheumatology/kex351.

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.

Keywords: computed tomography; pulmonary arterial hypertension; pulmonary veno-occlusive disease; systemic sclerosis; systemic sclerosis-pulmonary arterial hypertension.

Publication types

  • Evaluation Study

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Female
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / diagnostic imaging*
  • Lung / diagnostic imaging
  • Male
  • Middle Aged
  • Prognosis
  • Pulmonary Edema / diagnostic imaging
  • Pulmonary Edema / etiology
  • Pulmonary Veno-Occlusive Disease / diagnostic imaging*
  • Pulmonary Veno-Occlusive Disease / etiology
  • Scleroderma, Systemic / complications*
  • Tomography, X-Ray Computed / methods
  • Tomography, X-Ray Computed / statistics & numerical data*