The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy

Table 1

VWF-targeted pharmacotherapy. Specific and nonspecific VWF antagonists and mechanism of action. VWF: von Willebrand factor; GpIb: glycoprotein Ib; Fab: fragment antigen binding; TNF: tumor necrosis factor; MA: monoclonal antibody; Ref.: references.

Drugs	Mechanism of action	Ref.
Nonspecific VWF antagonists
Statins	Anti-inflammatory effect (?)	[94]
Heparin	Binds VWF-A1 domain + anti-inflammatory effect (?)	[95–97]
N-Acetylcysteine (NAC)	ADAMTS13-like activity	[98–100]
Anti-TNFα	Anti-inflammatory effect (?)	[93]
Corticosteroids	Anti-inflammatory effect (?)	[92]
Colchicine	Microtubule disruption	[32]
Aurintricarboxylic acid (ATA)	Inhibits VWF-GpIb interaction	[65]
Specific VWF antagonists
h6B4-Fab	MA: inhibits VWF-GpIb interaction	[103]
GPG-290	MA: inhibits VWF-GpIb interaction	[104]
AJvW-2	MA: inhibits VWF-GpIb interaction	[62]
AJW200	MA: inhibits VWF-GpIb interaction	[102, 105]
82D6A3	MA: inhibits VWF-collagen interaction	[107]
SZ-123	MA: inhibits VWF interaction with GpIb and collagen	[109]
ARC1779	Aptamers: inhibits VWF-GpIb binding	[111–113]
ARC15105	Aptamers: inhibits VWF-collagen binding	[114]
ALX-0081 (caplacizumab)	Nanobody: inhibits VWF-GpIb interaction	[117]
VCL	VWF recombinant fragment	[64]
BAX930	Recombinant ADAMTS13 (rADAMTS13)	[123]