Related Experiment Video
Updated: Jun 22, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Noninvasive detection of early pulmonary vascular dysfunction in scleroderma
F Reichenberger1, R Voswinckel, R Schulz
1University Giessen Lung Centre, University Hospital Giessen, Germany. frank.reichenberger@innere.med.uni-giessen.de
Background:
Pulmonary arterial hypertension is a serious complication in scleroderma and early diagnosis is warranted.
Patients And Methods:
33 Scleroderma patients (mean age 54 [SD 11] years) with normal systolic pulmonary artery pressure (sPAP), right and left ventricular parameters at rest were enrolled in this study. They were investigated by echocardiography during physical exercise and hypoxia. A hypertensive pulmonary vascular reaction was diagnosed when sPAP inappropriately increased during exercise compared to individual exercise tolerance, or if sPAP was >40mm Hg during hypoxia exposure, respectively.
Results:
In 17 patients there was a normal increase of sPAP at exercise of 34 [8]mm Hg (mean VO2 max 70 [17]% predicted), and 29 [5]mm Hg during hypoxia. Sixteen patients developed a hypertensive pulmonary vascular response with a sPAP of 47 [9]mm Hg during exercise (VO2max 64 [15]% pred.), and 54 [7]mm Hgmm Hg during hypoxia, respectively. The sPAP/VO2 max ratio was higher in patients with a hypertensive response (0.76 [0.17] vs. 0.49 [0.12] p<0.0001). There was no difference in scleroderma subtype, duration of disease, gender or age between groups. During a 3-year follow up one patient with a hypertensive response developed manifest pulmonary arterial hypertension.
Conclusion:
Non-invasive assessment of pulmonary circulation during physical exercise and/or hypoxia enables screening for pulmonary vascular dysfunction in scleroderma.
