Behçet’s disease: an update on pathogenesis, diagnosis and management of vascular involvement

Abstract

The objective of this review is to summarize reports of the prevalence, clinical presentation, diagnostic methodology and treatment of vasculitic manifestations of Behçet’s disease (BD). We performed a literature search on vasculitis in BD. Articles were selected which provided insight into the pathogenesis and clinical aspects of vasculitis. Vasculitis underlies many of the clinical features of BD. Small vessel vasculitis is often found in the pathology of the mucocutaneous manifestations of BD. Large vessel vasculitis has been reported in 15-40% of BD patients. Ultrasound, angiography and tomography are applied to confirm the diagnosis when venous involvement is suspected. Endothelial dysfunction plays a role in the pathogenesis of disease. Peripheral arterial involvement in BD occurs in the form of arterial occlusion or aneurysms. Pulmonary arterial involvement is often life-threatening. The cause of cardiac vascular involvement requires an aggressive diagnostic approach. Corticosteroids and immunosuppressive agents have been used successfully in the early stage of large vessel disease and should be used as an adjunct to surgery. An increasing amount of data is available regarding the role of anti-tumor necrosis factor (TNF) agents for the treatment of BD. Anticoagulant therapy may be hazardous in patients with aneurysmal dilatation of the pulmonary vascular tree and is not effective in the treatment of venous thrombosis. Inflammation of small and large vessels is very frequent in BD. Both arteries and veins may be involved. Early recognition and appropriate management of large vessel vasculitis in BD is essential to reduce associated morbidity and mortality

Methotrexate Therapy in Deep Venous Thrombosis in Behcet Disease in Children

“…Behcet vascular inflammation leads to stenosis, thrombosis and aneurisms. Most frequent vascular process is venous thrombosis, usually at lower extremity [26][27][28][29][30][31], as in our case, and this represents 85-93% [27] from vascular Behcet disease (the term vascular disease refers to patients that mostly present large blood vessels damage, often complicated with pulmonary aneurysms that may lead to death [32]). Secondly frequent thrombosis localization is cava vein [27], often reported in Mediterranean Basin and Europe [33].…”

“…Even if anticoagulation therapy is used in some cases (cardiac or cerebral manifestations) [22,51,52], except pulmonary aneurysm and it is encouraging even for children, most authors considers it inefficient [53][54][55][56][57][58] (thrombosis appears even if it is used anticoagulants alone). Although venous thrombosis is frequent, embolism is sporadic, which is why anticoagulants, antiplatelet and fibrinolytic therapy are not recommended [32,59,60]. Another reason to avoid these medications is high risk for bleeding, especially in symptomatic or asymptomatic pulmonary aneurysms [61].…”

Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?

“…The term “vasculo-Behçet,” which is included in the specific types of BD according to the Japanese criteria of 2003, is used in order to identify that subcategory of the patients with BD who have a tendency to develop large vessel lesions, often with an additive and progressive course [33]. The frequency of the vascular involvement among patients with BD ranges from 7.7 to 38% [18].…”

“…The lesions involve mainly the veins (29% of BD cases) and less frequently the arteries (8–16% of BD cases) and include vasculitis, thrombosis, and aneurysms [34]. Thrombosis represents the most usual manifestation, whereas the rupture of a pulmonary artery aneurysm consists the leading cause of death in vasculo-BD [33]. Nevertheless, the precise worldwide prevalences of large vein thrombosis in BD could not be recorded in this literature review because the majority of the international studies examine the large vessel involvement as a whole and they do not use a common and clear case definition.…”

Whether Behçet’s patients with large vessel involvement have concurrent small vessel involvement? A case-control Study

“…Small vessel involvement occurs in 48% to 90% of BD patients [7,8,9,10,11]. Moreover, vasculitis and phlebitis, lymphocytic vasculitis, and leukocytoclastic vasculitis have been reported in 48%, 31%, and 17% of skin biopsies in BD patients, respectively [12]. There is no data about the concurrence of large and small vessels Keywords: Behçet's disease; Vascular involvement; Nail fold capillaroscopy; Vasculitis Behçet's disease (BD) is a chronic multisystem disorder with the principal pathological finding of vasculitis, which may involve vessels of different sizes.…”