Pulmonary Veno-Occlusive Disease Affects Survival in PAH/Systemic Sclerosis

patient with diffuse systemic sclerosis
patient with diffuse systemic sclerosis
Having at least 2 features of pulmonary veno-occlusive disease demonstrated a trend toward worse survival in patients with pulmonary arterial hypertension and systemic sclerosis.

In patients with systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH), having at least 2 features of pulmonary veno-occlusive disease (PVOD), a rare form of pulmonary hypertension (PH), is associated with a reduced survival rate, according to study findings published in Rheumatology.

The investigators sought to examine whether computed tomography (CT) signs of PVOD (CTS-PVOD) were common in patients with SSc-PAH, and whether these signs were associated with pulmonary edema after initiating treatment and with worse survival outcomes.

A total of 66 patients with SSc and precapillary PH who had received chest CT scans were blindly scored by 2 radiologists for the presence of CTS-PVOD. All patients were divided into 2 groups based on the number of CTS-PVOD (≤1 or ≥2). The presence of pulmonary edema after initiation of therapy was established by radiographic review and case notes. The mean patient age was 63±12 years (range, 34-83 years), and the majority of patients (89%) were women.

Overall, 89% (59 of 66) of the patients had ≤1 CTS-PVOD and 11% (7 of 66) had ≥2

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CTS-PVOD. All patients who were evaluated received PAH-specific therapy; 29% of those individuals with ≥2 CTS- PVOD developed PAH while receiving treatment (P =.008). Pulmonary edema on treatment was fairly common in participants with ≥2 CTS-PVOD. Univariate analysis demonstrated that ≥2 CTS-PVOD were associated with a trend toward worse survival (hazard ratio, 2.57; 95% CI, 0.97-6.80; P =.06).

The researchers concluded that although CTS-PVOD occurred less often in this cohort of patients with SSc-PAH than noted in prior reports, the presence of at least 2 of these signs is associated with the development of pulmonary edema on treatment and worse survival.


Connolly MJ, Abdullah S, Ridout DA, Schreiber BE, Haddock JA, Coghlan JG. 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