Thrombosis in vasculitis: from pathogenesis to treatment

Abstract

In recent years, the relationship between inflammation and thrombosis has been deeply investigated and it is now clear that immune and coagulation systems are functionally interconnected.Inflammation-induced thrombosis is by now considered a feature not only of autoimmune rheumatic diseases, but also of systemic vasculitides such as Behçet’s syndrome, ANCA-associated vasculitis or giant cells arteritis, especially during active disease.These findings have important consequences in terms of management and treatment. Indeed, Behçet’syndrome requires immunosuppressive agents for vascular involvement rather than anticoagulation or antiplatelet therapy, and it is conceivable that also in ANCA-associated vasculitis or large vessel-vasculitis an aggressive anti-inflammatory treatment during active disease could reduce the risk of thrombotic events in early stages.In this review we discuss thrombosis in vasculitides, especially in Behçet’s syndrome, ANCA-associated vasculitis and large-vessel vasculitis, and provide pathogenetic and clinical clues for the different specialists involved in the care of these patients.

Neutrophil Activation Promotes Fibrinogen Oxidation and Thrombus Formation in Behçet Disease

“…15 In particular, neutrophils can mediate tissue injury in different ways: actually, neutrophils in BD display an intrinsic hyperactivation, probably HLAB*51-related, and usually participate in perivascular infiltration in BD lesions. 16 Based on these pathogenetic concepts and clinical experience, the European League Against Rheumatism (EULAR) recommendations for the management of BD suggest that thrombosis should be treated with immunosuppression rather than anticoagulation, 17 as an inflammation-induced thrombosis.…”

“…M2 (CD11b/CD18, Mac-1) and X2 (CD11c/CD18, p150,95) are the main fibrinogen receptors and are expressed on neutrophils, monocytes, macrophages and several subsets of lymphocytes. 20 In BD, endothelial cell dysfunction, increased reactive oxygen species (ROS) production, 21 and neutrophil hyperfunction have been reported, 15 together with an impaired fibrinolysis. 22 In this study, to highlight the mechanisms of inflammation-induced thrombosis, we investigated fibrinogen modifications, fibrin susceptibility to plasmin-lysis, plasma redox status, leukocyte oxidative stress, and possible ROS sources in a population of BD patients.…”

Anticoagulation of Behcet’s Disease with Thrombosis: A Series of 28 Cases in a Tertiary Care Centre in Saudi Arabia

“…Inflammation-induced thrombosis was considered a feature of BD especially during active disease. The pathophysiology of thrombosis in BD is unknown, but systemic inflammation seemed to play a major role whereas other thrombophilia factors were less relevant [19].…”

Vascular Manifestations of Behçet’s Disease

“…This led to the suggestion that these factors may be additional risk factors for thrombosis in certain people [33].…”