Activated protein C levels in Behçet's disease and risk of venous thrombosis

Abstract

SummaryBehçet'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), a 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, a 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 (0AE65 ± 0AE19 ng/ml, 86% ± 22% and 15AE5 ± 7AE1 ng/ml respectively) than in those without VTE (0AE78 ± 0AE17 ng/ml, 100% ± 15% and 22AE1 ± 15AE3 ng/ml) (P < 0AE05). In patients, APC levels below 0AE75 ng/ml (10th percentile of the control group) increased the risk of VTE about fivefold (odds ratio ¼ 5AE1; 95% confidence interval ¼ 1AE1-23AE4). These results show that reduced APC levels are associated with the high incidence of VTE in Behçet's disease.

Low Plasma Activated Protein C-Protein C Inhibitor Complex Concentration Is Associated with Vascular Access Failure in Hemodialysis Patients

“…In addition, patients with impaired kidney function have higher than normal levels of P-TM-ag. We were not able to confirm that postdialysis P-TM-ag levels were significantly elevated compared to predialysis levels [10,11,12,13] or that the P-TM-ag values were correlated with failure of the vascular access [14, 15]. Our results regarding P-TM-ag are supported by the fact that we also measured P-TM-act, which was not correlated with the failure of vascular access.…”

“…The P-APC-PCI complex is increased in hypercoagulable states such as deep vein thrombosis and in patients with myocardial infarction [13, 14]. However, it has been reported to be low in Behçet patients with repeated thromboses [15]. An elevated plasma homocysteine (P-Hcy) concentration is considered a risk factor for venous and arterial thromboembolism [16].…”

Venous thromboembolism in systemic autoimmune diseases: A narrative review with emphasis on primary systemic vasculitides

“…Specifically, patients with BD and a history of VTE had the lowest APC level. 63 In another study from Turkey, APC resistance level was significantly lower in 116 patients with BD when compared with 70 healthy controls. Thrombophlebitis was present in 56 patients (48.3%) out of 116 patients and 11 patients (9.5%) had DVT.…”

Síndrome Hughes-Stovin: Caso clínico

“…No se han descrito en estos pacientes alteraciones trombofílicas y se ha propuesto una mutación en el gen de la protrombina con niveles aberrantes de la proteína C 16,17 , lo que no está demostrado.…”