Abstract
OBJECTIVE—To investigate the expression of thrombomodulin in scleroderma associated pulmonary hypertension. METHODS—Soluble thrombomodulin (sTM), was measured in plasma samples from 34 scleroderma patients shown to have pulmonary hypertension at echocardiogram, and comparison drawn against samples from 38 scleroderma control patients, and 20 healthy controls. Serial measurements of sTM were performed in the 34 patients with scleroderma associated pulmonary hypertension to investigate possible changes in sTM concentration with progression of the condition. RESULTS—Mean sTM was raised in scleroderma associated pulmonary hypertension when compared with scleroderma controls (mean sTM 65.4 ng/ml v 43.3 ng/ml, p<0.05), and when compared with healthy controls (mean sTM 38.1 ng/ml, p<0.05). There was no significant difference between mean sTM in scleroderma controls and healthy controls. Mean sTM concentration did not change with progression of pulmonary hypertension. CONCLUSION—Plasma sTM is raised in scleroderma associated pulmonary hypertension. The pathogenesis of scleroderma associated pulmonary hypertension may be distinct from the pathogenesis of other forms of pulmonary vascular disease.
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Figure 1 .
Plasma soluble thrombomodulin concentration against patient group. Solid bars indicated mean soluble thrombomodulin concentration. * p<0.05 versus scleroderma and healthy control values.
Figure 2 .

Progression of pulmonary hypertension over a two year period in 34 patients with scleroderma associated pulmonary hypertension. * p<0.05, ** p<0.001 versus baseline measurement of pulmonary artery pressure. Serial measurements of sTM showing no significant change over two year follow up.
Selected References
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