[2008] [SAT0220] EARLY SCREENING FOR PULMONARY HYPERTENSION IN SCLERODERMA

F. Reichenberger¹, R. Voswinckel¹, A. Ghofrani¹, H. Olscheski², W. Seeger^{3 1}University Giessen Lung Center, University Hospital Giessen, Giessen, Germany; ²Department of Pneumonology, Medical University, Graz, Austria; ³University Giessen Lung Center, University Hospital Giessen, Graz, Germany

Background: Pulmonary arterial hypertension is a serious complication in scleroderma and early diagnosis is warranted.
Objectives: We used a new screening system for detection of exercise induced pulmonary hypertension.
Methods: We non-invasively evaluated pulmonary circulation using stress echocardiography to detect pulmonary vascular dysfunction in 33 scleroderma patients (mean age 54 [SD 11] years). All patients had normal systolic pulmonary artery pressure (sPAP) and left ventricular parameters at rest. A hypertensive pulmonary vascular reaction has been diagnosed when sPAP inappropriately increased during exercise, or sPAP was > 40 mmHg 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 Hg during hypoxia, respectively. This was invasively confirmed in 3 patients. The sPAP/VO2 max ratio was higher in patients with 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.
Conclusion: Non-invasive assessment of pulmonary circulation during stress enables screening for early pulmonary hypertension in scleroderma.

Ann Rheum Dis 2008;67(Suppl II):503

Scleroderma, Myositis and related syndromes