Background Systemic Sclerosis (SSc) patients were recently described to have a higher risk of pulmonary embolism (PE) than healthy individuals¹. Portuguese expert consensus² recommends screening for PE when respiratory distress cannot be explained by the presence of cardiovascular or pulmonary interstitial involvement (ILD).

Objectives We present our 5-year experience with PE in SSc patients.

Methods We included all adult SSc patients submitted to lung ventilation/perfusion scintigraphy (V/Q scan) or CT pulmonary angiography (AngioCT) due to worsening dyspnea/fatigue or isolated reduction of the carbon monoxide diffusing capacity (DLCO), from January 2010 to December 2014. We collected demographic data, functional class according to the World Health Organization (WHO-FC) classification, comorbidities, risk factors for PE and results from AngioCT and V/Q scans. A descriptive statistical analysis was performed.

Results Eighteen patients were included: 15 females, with a mean age of 55±14 years and median disease duration 2 (IQR 2.5) years. Nine patients were diagnosed with PE, 8 of which by V/Q scan and 1 using AngioCT. Eight patients had bilateral peripheral multisegmental PE and 1 unisegmental PE. Five PE patients had a WHO-FC of 1-2. Three of the remaining 4 patients with WHO-FC >2 had concomitant extensive ILD, and one patient had pulmonary hypertension. No patient had thrombophilia, previous surgery or cancer. The frequency of hypertension, diabetes, smoking, obesity and dyslipidemia was similar to patients without PE. Anti-topoisomerase positive SSc patients (Anti-topo+) were slightly overrepresented in the PE group (5 vs 2 Anti-topo+ patients without PE). Other disease imunophenotypes were equally distributed between subgroups.

Conclusions Oligosymptomatic PE is common in SSc patients. Traditional procoagulant risk factors do not account for this increased risk. Disease-specific risk factors, such as vascular endothelial dysfunction, may be involved. The impact of PE in disease prognosis is yet unknown.

References

1. Chung WS, Lin CL, Sung FC, Hsu WH, Yang WT, Lu CC, Kao CH. Systemic sclerosis increases the risks of deep vein thrombosis and pulmonary thromboembolism: a nationwide cohort study. Rheumatology (Oxford). 2014 Sep;53(9):1639-45. doi: 10.1093/rheumatology/keu133. Epub 2014 Apr 8.

2. Grupo de Estudos de Doenças Reumáticas Sistémicas (GEDRESIS). Manual Prático de Esclerose Sistémica. Sociedade Portuguesa de Reumatologia; 2014.

Disclosure of Interest None declared