Predicting Mortality in Systemic Sclerosis-PAH With CT Signs of Veno-Occlusive Disease

pulmonary artery hypertension
pulmonary artery hypertension
A blinded analysis sought to determine whether CT signs of pulmonary veno-occlusive disease are common and predictive of mortality in patients with systemic sclerosis and pulmonary arterial hypertension.

Radiological signs of pulmonary veno-occlusive disease (PVOD) are less common than previously reported in patients with systemic sclerosis-pulmonary arterial hypertension (SSc-PAH), but the presence of at least two of these signs is associated with reduced survival, according to a retrospective analysis published in Rheumatology.1

Radiologists at a center that manages more than 500 patients with SSc-PAH blindly scored computed tomography (CT) thorax scans for signs of PVOD in 66 patients with SSc and precapillary pulmonary hypertension. They found that 59 patients (89%) had one or fewer CT signs of PVOD while 7 patients (11%) had ≥2 CT signs of PVOD. These findings are in contrast to a previous study that reported a much higher incidence with 62% of patients with SSc-PAH having ≥2 CT signs of PVOD.2

Following a diagnosis of PAH, all 66 patients received PAH-specific therapy and were followed until death or date of censorship. The overall incidence of pulmonary edema on PAH-specific therapy was low (6%); however, 3% of patients with ≤1 CT signs of PVOD developed pulmonary edema compared with 29% of patients with ≥2 CT signs of PVOD (P =.008).

Patients with at ≥2 CT signs of PVOD also had a trend to a more rapid progression to death compared with patients with ≤1 CT signs of PVOD and a mean non-adjusted survival rate of 2.8 years vs 5.9 years, respectively. The lack of a significant effect may be explained by the study being underpowered.   

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The investigators concluded by saying that the CT signs of 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 edema on therapy and a trend towards worse survival on univariate analysis.”1


  1. Connolly MJ, Abdullah S, Ridout DA, et al. Prognostic significance of computed tomography criteria for pulmonary veno-occlusive disease in systemic sclerosis-pulmonary arterial hypertension [published online September 26, 2017]. Rheumatology (Oxford). doi:10.1093/rheumatology/kex351
  2. Günther S, Jaϊs X, Maitre S, et al. Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension. Arthritis Rheum. 2012;64:2995-3005.