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. 2017 Jul 14;23(26):4796-4805.
doi: 10.3748/wjg.v23.i26.4796.

Levels and Activities of Von Willebrand Factor and Metalloproteinase With Thrombospondin type-1 Motif, Number 13 in Inflammatory Bowel Diseases

Free PMC article

Levels and Activities of Von Willebrand Factor and Metalloproteinase With Thrombospondin type-1 Motif, Number 13 in Inflammatory Bowel Diseases

Dorota Cibor et al. World J Gastroenterol. .
Free PMC article


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.


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 < 0.7 and vWF: CB/vWF:Ag < 0.7 in patients with ulcerative colitis, Crohn’s disease and in the control group.
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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    1. Murthy SK, Nguyen GC. Venous thromboembolism in inflammatory bowel disease: an epidemiological review. Am J Gastroenterol. 2011;106:713–718. - PubMed
    1. Novacek G, Weltermann A, Sobala A, Tilg H, Petritsch W, Reinisch W, Mayer A, Haas T, Kaser A, Feichtenschlager T, et al. Inflammatory bowel disease is a risk factor for recurrent venous thromboembolism. Gastroenterology. 2010;139:779–787, 787.e1. - PubMed
    1. Dhillon AP, Anthony A, Sim R, Wakefield AJ, Sankey EA, Hudson M, Allison MC, Pounder RE. Mucosal capillary thrombi in rectal biopsies. Histopathology. 1992;21:127–133. - PubMed
    1. Owczarek D, Cibor D, Głowacki MK, Rodacki T, Mach T. Inflammatory bowel disease: epidemiology, pathology and risk factors for hypercoagulability. World J Gastroenterol. 2014;20:53–63. - PMC - PubMed
    1. Thompson NP, Wakefield AJ, Pounder RE. Inherited disorders of coagulation appear to protect against inflammatory bowel disease. Gastroenterology. 1995;108:1011–1015. - PubMed