Background Risk of pulmonary embolism (PE) in systemic sclerosis (SSc) has been estimated between 2.51–3.47 fold higher when compared to non-SSc patients (pts). Proinflammatory state, vasculopathy and vascular injury may contribute to a prothrombotic state in SSc and increased risk for venous thromboembolism.
Objectives Calculate frequency and identify possible risk factors for PE among SSc pts;analyse efficacy of longterm anticoagulation (ACO) in these pts.
Methods We conducted a retrospective analysis of 110 pts with SSc followed in our Rheumatology department and selected those who performed lung ventilation/perfusion scintigraphy (V/Q scan) or CT pulmonary angiography due to worsening dyspnea/fatigue or isolated reduction of the carbon monoxide diffusing capacity (DLCO). We collected demographic features, comorbidities, age at SSc diagnosis, anti-nuclear antibody specificities, dyspnea according to New York Heart Association (NYHA) classes and results of cardiopulmonary exams. PE and related variables were assessed at baseline and after 12 months of ACO.
Results PE was diagnosed in 12 out of 29 (41.4%) SSc pts that met inclusion criteria, with the majority presenting bilateral peripheral multisegmental defects. Most were females (91.7%), mean age of 59.4 (±12.7) years (yrs). Two thirds were diagnosed with limited SSc with mean disease duration of 13.3 (±12.9) yrs.
Mean time between SSc and PE diagnosis was 8.5±8.2 yrs, although one third of the pts was diagnosed within the first year of SSc diagnosis. One patient was taking oral contraceptives and none had thrombophilia, previous surgery or cancer.
One third was classified as having NYHA class≥3, with a mean N-terminal pro-brain natriuretic peptide (NTproBNP) of 1108pg/mL (19 to 8069). Six pts had concomitant interstitial lung disease (ILD) and 8 had an estimated pulmonary artery systolic pressure (PASP)≥35 mmHg (6 of them had concomitant ILD). From these only 2 had pulmonary hypertension confirmed by right heart catheterization and 1 died.
When comparing SSc pts with and without PE, Scl70 positivity was more common in pts with PE (p=0.041). No significant associations were found between PE and several cardiovascular risk factors.
From the 12 pts with PE, 10 were on longterm ACO:5 on rivaroxaban, 4 on warfarin and 1 on apixaban. Clinical reassessment after 12 months of ACO is shown in figure 1.
Conclusions Our results suggest that PE is frequent in SSc and must be considered in the differential diagnosis of worsening fatigue and dyspnea and/or reduction of DLCO/PASP increase. PE may occur more frequently in Scl70 positive pts and early in disease course, probably due to vasculopathy and vascular injury being more prominent in the early phase of the disease. Although there is no consensus regarding the optimal ACO, the disease's vasculopathy seems to be an important contributor, potentially preventing improvement in perfusion defects, regardless of the anticoagulant used.
Disclosure of Interest None declared