Levels and activities of von Willebrand factor and metalloproteinase with thrombospondin type-1 motif, number 13 in inflammatory bowel diseases

Dorota Cibor, Danuta Owczarek, Saulius Butenas, Kinga Salapa, Tomasz Mach, Anetta Undas, Dorota Cibor, Danuta Owczarek, Saulius Butenas, Kinga Salapa, Tomasz Mach, Anetta Undas

Abstract

Aim: To evaluate the levels of von Willebrand factor (VWF) and metalloproteinase with thrombospondin type-1 motif, number 13 (ADAMTS13) in inflammatory bowel disease (IBD) and correlate them with the disease activity.

Methods: Consecutive patients with IBD aged 18 years or older were enrolled in the study. Forty-seven patients with ulcerative colitis (UC), 38 with Crohn's disease (CD), and 50 healthy controls were included. The white blood cell count, haematocrit, platelet count, fibrinogen, partial activated thromboplastin time, C-reactive protein, albumin, VWF antigen level (VWF:Ag), VWF ristocetin cofactor activity (VWF:RCo), VWF collagen-binding activity (VWF:CB), and ADAMTS13 antigen level (ADAMTS13:Ag) and activity (ADAMTS13act) were measured. The following ratios were assessed: VWF:RCo/VWF:Ag, VWF:CB/VWF:Ag, VWF:Ag/ADAMTS13act, and ADAMTS13act/ADAMTS13:Ag.

Results: Compared to controls, the odds ratio (OR) of an elevated VWF: Ag > 150% was 8.7 (95%CI: 2.7-28.1) in the UC group and 16.2 (95%CI: 4.8-54.0) in the CD group. VWF:CB was lower in UC patients, and active CD was associated with a higher VWF: RCo (+38%). The ORs of VWF:CB/VWF:Ag < 0.7 (a marker of acquired von Willebrand syndrome) in the UC and CD groups were 11.9 (95%CI: 4.4-32.4) and 13.3 (95%CI: 4.6-38.1), respectively. Active UC was associated with lower ADAMTS13:Ag (-23%) and ADAMTS13act (-20%) compared to UC in remission. Patients with active CD had a 15% lower ADAMTS13act than controls. The activity of UC, but not that of CD, was inversely correlated with ADAMTS13:Ag (r = -0.76) and ADAMTS13act (r = -0.81).

Conclusion: Complex VWF-ADAMTS13-mediated mechanisms disturb haemostasis in IBD. A reduced WVF:CB is a risk factor for bleeding, while a lower ADAMTS13 level combined with an elevated VWF:Ag could predispose one to thrombosis.

Keywords: ADAMTS13; Acquired von Willebrand syndrome; Inflammatory bowel disease; Thrombosis; von Willebrand factor.

Conflict of interest statement

Conflict-of-interest statement: The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Activity of vWF: RCo in the Crohn’s disease group and the controls. CD: Crohn’s disease.
Figure 2
Figure 2
Occurrence of vWF:Ag > 150%, vWF:RCo/vWF: Ag

Figure 3

Correlation between selected haemostatic parameters…

Figure 3

Correlation between selected haemostatic parameters and disease activity, and its duration. A: Correlation…

Figure 3
Correlation between selected haemostatic parameters and disease activity, and its duration. A: Correlation between disease duration and vWF: CB in patients with UC; B: Correlation between disease activity and vWF: CB in patients with ulcerative colitis (UC); C: Correlation between disease activity and ADAMTS13:Ag in patients with UC. D: Correlation between disease activity and ADAMTS13act in patients with UC.
Figure 3
Figure 3
Correlation between selected haemostatic parameters and disease activity, and its duration. A: Correlation between disease duration and vWF: CB in patients with UC; B: Correlation between disease activity and vWF: CB in patients with ulcerative colitis (UC); C: Correlation between disease activity and ADAMTS13:Ag in patients with UC. D: Correlation between disease activity and ADAMTS13act in patients with UC.

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