Activated protein C levels in Behçet's disease and risk of venous thrombosis.
Behçet's disease is a multi-systemic inflammatory disorder of unknown cause. Most abnormalities have been associated with endothelial injury caused by vasculitis. Thrombosis occurs in about 25% of patients, although the mechanism is unknown. The objective of this study was to evaluate the protein C activation system in Behçet's disease and its correlation with venous thromboembolism (VTE). Thirty-nine patients (12 with VTE) and 78 age- and sex-matched controls were included in the study, and levels of protein C, protein S, activated protein C (APC), protein C inhibitor (PCI), soluble thrombomodulin (TM), antithrombin (AT), alpha(1)-antitrypsin, fibrinogen, factor VIII, von Willebrand factor (VWF) and C-reactive protein (CRP) were measured. APC and TM levels were significantly lower in patients than in controls, whereas protein S, AT, alpha(1)-antitrypsin, fibrinogen, factor VIII, VWF and CRP levels were significantly higher in patients than in controls. APC, PCI and TM levels were lower in patients with VTE (0.65 +/- 0.19 ng/ml, 86% +/- 22% and 15.5 +/- 7.1 ng/ml respectively) than in those without VTE (0.78 +/- 0.17 ng/ml, 100% +/- 15% and 22.1 +/- 15.3 ng/ml) (P < 0.05). In patients, APC levels below 0.75 ng/ml (10th percentile of the control group) increased the risk of VTE about fivefold (odds ratio = 5.1; 95% confidence interval = 1.1-23.4). These results show that reduced APC levels are associated with the high incidence of VTE in Behçet's disease.
Navarro
Silvia
S
Research Centre, Hospital Universitario La Fe, Valencia, Spain.
Ricart
José María
JM
Medina
Pilar
P
Vayá
Amparo
A
Villa
Piedad
P
Todolí
José
J
Estellés
Amparo
A
Micó
María Luisa
ML
Aznar
Justo
J
España
Francisco
F
D000293:Adolescent; D000328:Adult; D001528:Behcet Syndrome / Q000097:blood* / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D001777:Blood Coagulation; D001779:Blood Coagulation Factors / Q000032:analysis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011486:Protein C / Q000032:analysis*; D013923:Thromboembolism / Q000097:blood / Q000209:etiology; D019851:Thrombophilia / Q000097:blood / Q000209:etiology; D020246:Venous Thrombosis / Q000097:blood* / Q000209:etiology
D001779:Blood Coagulation Factors; D011486:Protein C