Protein C System in Patients with Idiopathic Pulmonary Fibrosis—Preliminary Report

Abstract

Introduction: 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). Material and methods: Study grup 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. Results: 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. Conclusions: 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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“…Protein S can be found in plasma in a free form or complexed to C4BP (C4b-binding protein) [ 79 , 80 ]. In a study on 33 IPF patients and 44 controls, no significant differences were observed in the circulating levels of free and total protein S in IPF patients compared with healthy individuals [ 81 ], while in another study, lower plasma concentrations of protein S were detected in a small cohort of 11 patients affected by IPF if compared to 20 healthy controls [ 82 ]. More recently, it was reported that 3,5,3′-triiodothyronine (T3) administration in a mice model of pulmonary fibrosis improved alveolar regeneration, in a process involving protein S-TAM signaling [ 83 ].…”