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Table 1 Summary of clinical and therapeutic features of thrombotic events in systemic vasculitis

From: Thrombosis in vasculitis: from pathogenesis to treatment

Disorders District involvement and treatment Data based on References
Behçet’s syndrome Venous involvement - venous involvement is common and makes up 75% of all vascular complications. Venous thrombosis occurs more frequently in males with active disease during the early years, sometimes at the onset of disease, and tends to recur. Large survey (387 pts) and retrospective evaluation (2319 pts) [6,38-41]
Arterial involvement - the most characteristic arterial manifestations are aneurysms whereas arterial thrombosis is less common. The coexistence of thrombosis and aneurysms is a peculiar feature of Behçet. Retrospective evaluations and expert experience [6,11,13,42-44]
Treatment - the management of vascular thrombosis is based on immunosuppressants rather than anticoagulants. Azathioprine and cyclosporine in association with low dose corticosteroids are usually the first choice in the treatment of deep vein thrombosis and superficial vein thrombosis, while cyclophosphamide is the suggested treatment for arterial involvement. In resistant cases anti-TNFα agents could be considered. European League Against Rheumatism recommendations, large monocentric experience (64 pts) [8,13,45-51]
ANCA-associated vasculitis Venous involvement - increased incidence of venous thromboembolism, especially during active disease. Multicentric randomized placebo-controlled trial (180 pts), retroprospective analysis (up to 1130 pts) and monocentric experience (19 pts) [61,69-74]
Arterial involvement - increased incidence of arterial involvement and particularly of cardiovascular events. Two large retrospective studies (113 and 239 pts respectively) [75-77]
Treatment - there are controversial data on the use of statins, while there are no significant data on the use of antiplatelet and/or anticoagulant therapy. In vitro study, case reports [78-80]
Large-vessel vasculitis Venous involvement - increased risk of venous thromboembolism, both deep vein thrombosis and pulmonary embolism, in particular during the first year after diagnosis. Similar data are reported in polymyalgia rheumatica. Large population-based study (909 pts) and nationwide population study (535.538 individuals) [87-90]
Arterial involvement - increased risk of cardiovascular events, especially in giant cell arteritis. Large cohort study (3500 pts) and retrospective analysis (210 pts) [91-100]
Treatment - the use of antiplatelet/anticoagulant therapy is not effective for primary prophylaxis, whilst it could be beneficial as combination therapy with corticosteroids in established giant cell arteritis. In Takayasu disease the use of antiplatelet treatment could be protective for ischemic events. Cumulative meta-analysis (6 retrospective studies, 914 pts), monocentric retrospective evaluation (48 pts), retrospective analysis (297 pts) [101-104]