Background . Pulmonary arterial hypertension (PAH) is the main cause of morbimortality in systemic sclerosis (SSc). Increased Eng expression has been demonstrated in SSc patients. Objective . Ascertaining serum levels of Eng in SSc patients with and without elevated systolic pulmonary arterial pressure (sPAP) and comparing them with that of healthy volunteers. Methods . A cross-sectional study was carried out. A commercial ELISA kit was used for measuring serum concentrations of Eng in 60 subjects: 40 patients with SSc with and without elevated sPAP, compared to 20 healthy control subjects. Elevated sPAP was detected by echocardiogram. Results . No association between positive Eng and elevated sPAP was found when compared to the SSc without elevated sPAP group (<mml:math xmlns:mml="http://www.w3.org/1998/Math/MathML"><mml:mrow><mml:mtext>OR</mml:mtext><mml:mo>=</mml:mo><mml:mn>2.85</mml:mn></mml:mrow></mml:math>; 0.65–12.88 95% CI;<mml:math xmlns:mml="http://www.w3.org/1998/Math/MathML"><mml:mrow><mml:mi>P</mml:mi><mml:mo>=</mml:mo><mml:mn>.11</mml:mn></mml:mrow></mml:math>); however, an association was found between positive Eng and elevated sPAP compared to healthy controls (<mml:math xmlns:mml="http://www.w3.org/1998/Math/MathML"><mml:mrow><mml:mtext>OR</mml:mtext><mml:mo>=</mml:mo><mml:mn>23.22</mml:mn></mml:mrow></mml:math>; 2.46–1050.33 95% CI;<mml:math xmlns:mml="http://www.w3.org/1998/Math/MathML"><mml:mrow><mml:mi>P</mml:mi><mml:mo>=</mml:mo><mml:mn>.001</mml:mn></mml:mrow></mml:math>), and weak association was found between the positive Eng with SSc without elevated sPAP group compared to healthy controls (<mml:math xmlns:mml="http://www.w3.org/1998/Math/MathML"><mml:mrow><mml:mtext>OR</mml:mtext><mml:mo>=</mml:mo><mml:mn>8.14</mml:mn></mml:mrow></mml:math>, 0.8–393.74 95% CI;<mml:math xmlns:mml="http://www.w3.org/1998/Math/MathML"><mml:mrow><mml:mi>P</mml:mi><mml:mo>=</mml:mo><mml:mn>.046</mml:mn></mml:mrow></mml:math>). Conclusion . Raised serum levels of Eng in SSc patients compared to healthy controls were found, suggesting a role for Eng in SSc vasculopathy and not just in elevated sPAP. However, prospective studies are needed to verify such observations.