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

TL;DR

This study found that Behçet’s Disease patients have lower ADAMTS-13 activity and higher von Willebrand Factor levels compared to healthy individuals, but these changes are not linked to vascular involvement.

Contribution

The study reveals altered ADAMTS-13 and VWF levels in Behçet’s Disease, suggesting a potential role in thrombosis mechanisms.

Findings

BD patients had lower ADAMTS-13 antigen, inhibitor, and activity compared to healthy controls.

Von Willebrand Factor levels were higher in BD patients.

ADAMTS-13 activity was higher in BD patients with vascular involvement, but VWF levels were similar between subgroups.

Abstract

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.

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.

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.

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.

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