Behçet’s Disease as a Model of Venous Thrombosis

Abstract

Behçet’s disease (BD) is a chronic inflammatory disease of unknown aetiology characterized by recurrent oral, genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in BD. The frequency of vascular lesions in BD, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. In this review, various factors of thrombogenesis in BD, particularly pro- and antithrombotic endothelial and non-endothelial factors, factors of coagulation, platelet activation and rheological changes are presented and discussed from positions of Virchow’s triad of venous thrombosis. Despite advances in understanding of thrombogenesis in BD, still many issues of diagnosis and targeted preventive and therapeutic measures remain unresolved. Further studies are needed to clarify the pathobiology of BD-related thrombosis and to provide the clinicians with recommendations over the utility, safety and effectiveness of the antithrombotic therapy in BD.

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

“…Many antiphospholipid antibodies were tested in BD. Elevated levels of anticardiolipin antibodies were associated with retinal and cerebrovascular pathology in BD [15]. In study from Turkey, among 96 patients with thrombosis, patients with recurrent thrombotic events had a higher incidence of thrombophilias than those patients with only single thrombotic event [16].…”

Vascular Manifestations of Behçet’s Disease

“…A significant number of patients have elevated levels of IgG aCL, but they are not associated with venous or arterial thrombosis. No correlation was found between any variable and other clinical manifestations of the disease [15,16].…”

Venous Thrombosis in Behcet’s Disease

“…However, in general immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide or cyclosporine are recommended for the treatment of venous thrombosis in BD. There is no enough evidence of benefit with anticoagulants or fibrinolytic agents in the management of thrombosis of BD (La Regina et al, 2010). Further studies are needed to clarify the safety and effectiveness of antithrombotic therapy in BD.…”

“…Deep vein thrombosis is seen in about one-fifth of Turkish patients with BD (Gul et al, 1999). Lower extremities is the most frequent site of thromboses but thromboses of other venous sites such as superior and inferior vena cava, coronary, portal, renal and pulmonary veins have been identified (Houman et al, 2001;La Regina et al, 2010). Leg ulcers in BD, which may be caused by vasculitis or deep vein thrombosis, have a chronic recurrent course and are refractory to treatment (Jung et al, 2008;Kartal Durmazlar et al, 2008b;.…”

“…Some studies have shown hyperhomocysteinemia as a correctable risk factor for thrombosis in BD (Kartal Durmazlar et al, 2008aOmar et al, 2007;Ozdemir et al, 2004;Er et al, 2002;Sarican et al, 2007). In a recent work, thrombogenesis in BD is discussed through the concept of Virchow's triad of venous thrombosis (La Regina et al, 2010). Based on this concept; abnormal blood flow, abnormal vessel wall, abnormal blood constituents are presented in Table 3.…”