Plasma levels of molecular markers of blood coagulation and fibrinolysis in progressive systemic sclerosis (PSS)

Abstract

Seventy-four patients with PSS were evaluated with regard to plasma concentration of blood coagulation and fibrinolysis factors: fibrinogen (Fbg), prothrombin time (PT), active partial thromboplastin time (APTT), protein C, thrombin-antithrombin III complex (TAT), antithrombin-III (AT-III), factor XIII (XIII) fibrinopeptide A (FPA), α 1-antitripsin ( α 1-AT), plasminogen (Pmg), α 2-plasmin inhibitor plasmin complex (PIC), α 2-plasmin inhibitor ( α 2-PI), α 2-macroglobulin ( α 2-MG), fibrinopeptide B β 15–42 (FPB β 15–42) and soluble fibrin monomer complex (SFMC), FDP (fibrin degradation product) and D-dimer. They were also evaluated with regard to platelet-derived proteins: β-thromboglobulin (β-TG), platelet factor 4 (PF4), thromboxane B 2 and 6-keto-prostaglandin F 1α (6KF). In the coagulation/fibrinolysis systems high plasma levels of TAT, AT-III, FPA, α 2-MG and FPB β 15–42 could be demonstrated in more than 50% of total PSS patients. There was no statistical correlation between those of TAT and AT-III. Plasma levels of PIC, D-dimer, FDP and SFMC were not always high. There was no statistical correlation between those of TAT and PIC. These data lead us to consider that α 2-MG may play an important role for inhibiting PIC, which accelerates the conversion from fibrin into FDP. Subsequently, there were high plasma levels of FPB β 15–42 converted from fibrin monomer. These data seem to be indicative of an involvement of coagulation and platelet disorder in PSS. These platelet-vessel system disorders might be closely related to the pathophysiology of PSS.