Abstract
The natural anticoagulant-activated protein C system plays an important role in the pathogenesis of idiopathic pulmonary fibrosis. The purpose of this study was to evaluate the concentration of protein C (PC), protein S (PS), thrombomodulin (TM), selectin E (sSelE), and thrombin-antithrombin complex (TAT) in patients with idiopathic pulmonary fibrosis (IPF). Study group consisted of 11 patients aged 51.5 +/- 8.62 years with idiopathic pulmonary fibrosis and 20 healthy adults as control. Concentration of PC, PS TM, sSelE and TAT in plasma with ELISA method was assessed. We observed significantly lower plasma concentration of PC (98.24 +/- 16.17% vs. 130.59 +/- 19.03%), PS (71.31 +/- +/- 12.95% vs. 93.47 +/- 18.63%), TM (2.67 +/- 0.40 ng/ml vs. 3.99 +/- 1.16 ng/ml) and significantly higher level of TAT complex (Me = 4.00 mg/ml vs. 2.20 mg/ml) and sSelE (Me = 36.40 ng/ml vs. 22.84 ng/ml) in patients with idiopathic pulmonary fibrosis as compared to controls. In presented pilot study we observed decreased activity of protein C system and increased thrombin generation in peripheral blood of patients with idiopathic pulmonary fibrosis.
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