Noninvasive detection of early pulmonary vascular dysfunction in scleroderma.
BACKGROUND
Pulmonary arterial hypertension is a serious complication in scleroderma and early diagnosis is warranted.


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.


CONCLUSIONS
Non-invasive assessment of pulmonary circulation during physical exercise and/or hypoxia enables screening for pulmonary vascular dysfunction in scleroderma.
Reichenberger
F
F
University Giessen Lung Centre, University Hospital Giessen, Germany. frank.reichenberger@innere.med.uni-giessen.de
Voswinckel
R
R
Schulz
R
R
Mensch
O
O
Ghofrani
H A
HA
Olschewski
H
H
Seeger
W
W
D018450:Disease Progression; D015150:Echocardiography, Doppler; D004730:Endothelium, Vascular / Q000000981:diagnostic imaging* / Q000503:physiopathology; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000209:etiology / Q000503:physiopathology; D000860:Hypoxia / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010101:Oxygen Consumption; D011379:Prognosis; D011651:Pulmonary Artery / Q000000981:diagnostic imaging* / Q000503:physiopathology; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology; D016896:Treatment Outcome