Plasma Levels of ADAMTS-13 Antigen, ADAMTS-13 Inhibitor, ADAMTS-13 Activity, and Von Willebrand Factor in Patients with Behçet's Disease.

Background: Behçet's Disease (BD) is characterised by recurrent aphthous oral and genital ulcers. Vascular involvement is one of the poor prognostic factors. Previously, von Willebrand Factor (VWF) has been detected higher in BD compared with healthy controls. We hypothesised that decreased activity or increased inhibitor levels of ADAMTS-13 may cause increased levels of VWF. Therefore, we investigated ADAMTS-13 in patients with BD.

Methods: We included in total of 42 patients with BD and 41 healthy controls (HCs) in this cross-sectional study. Parametric data with normal distribution were compared with Student's t-test and ANOVA, and nonparametric data with non-normal distribution were compared with Mann Whitney U and Kruskal Wallis tests.

Results: The patients showed lower ADAMTS-13 antigen, lower ADAMTS-13 inhibitor, lower ADAMTS-13 activity, and higher VWF levels compared with HCs. ADAMTS-13 activity was higher in vascular involvement compared with non-vascular involvement (18.26 ± 7.3 vs 12.05 ± 6.49, p=0.012). VWF levels were also similar between vascular and non-vascular subgroups.

Conclusion: Reduced ADAMTS-13 activity and increased VWF levels were detected in BD. This change has not been seen in vascular BD. The underlying pathogenetic mechanisms seem more complex in the formation of thrombosis.