Thrombosis in Vasculitis: from Pathogenesis to Treatment
Emmi et al. Thrombosis Journal (2015) 13:15 DOI 10.1186/s12959-015-0047-z REVIEW Open Access Thrombosis in vasculitis: from pathogenesis to treatment Giacomo Emmi1*†, Elena Silvestri1†, Danilo Squatrito1, Amedeo Amedei1,2, Elena Niccolai1, Mario Milco D’Elios1,2, Chiara Della Bella1, Alessia Grassi1, Matteo Becatti3, Claudia Fiorillo3, Lorenzo Emmi2, Augusto Vaglio4 and Domenico Prisco1,2 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. Keywords: Inflammation-induced thrombosis, Thrombo-embolic disease, Deep vein thrombosis, ANCA associated vasculitis, Large vessel vasculitis, Behçet syndrome Introduction immunosuppressive treatment rather than anticoagulation The relationship between inflammation and thrombosis is for venous or arterial involvement [8], and perhaps one not a recent concept [1], but it has been largely investigated might speculate that also in AAV or LVV an aggressive only in recent years [2].
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