AB1268 VASCULAR INVOLVEMENT IN BEHÇET’S DISEASE : ABOUT 87 CASES
Authors: Aya Fraj, Raja Elamri, wafa garbouj, Zainab Alaya, Rim Khalfallah, Heyfa Tounsi, Imen Chaabane
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Behçet's disease (BD) is a systemic vasculitis which ethiopathogenesis still poorly understood. Vascular involvement is one of the major causes of mortality and morbidity in this disease. It occupies a special place in the cardiovascular pathologies because of its venous and arterial tropism all confounded caliber but also because of its pejorative prognosis as well on the functional level as vital. <h3>Objectives:</h3> Our goal was to present the various venous and arterial manifestations of BD, their epidemiological, clinical, evolutionary and therapeutic characteristics. <h3>Methods:</h3> This is a retrospective study over a period of 9 years [2009-2018] that enrolled 87 patients who met the international criteria for Behçet's disease. The characteristics of vascular involvement were studied. <h3>Results:</h3> Twenty-two patients had vascular involvement (25%). There are 17 men and 5 women (sex ratio H/F=3,4), mean age at diagnosis of angina Behçet 39 years [22 years-63 years]. The vascular lesions were indicative of the disease in 17 cases (77%), and for the other 5 patients, the mean time to onset of vascular disease compared with the diagnosis of BD was 2 years. Venous thrombosis involved 20 patients (90%). They were deep in 16 patients, superficial in one patient and superficial and deep in 3 patients. It was recurrent in 14 patients. The most common site of deep vein thrombosis was the lower limbs (n=17). The others had unusual sites: inferior vena cava thrombosis (n = 3), superior vena cava thrombosis (n = 2), sus-hepatic vein (n=1) and upper limb thrombosis (n = 1). Pulmonary embolism was noted in one case. Arterial damage involved 4 patients (20%) divided into arterial aneurysms (3 cases) and arterial thromboses (2 cases). The locations were divided as follows: lower extremity arteries in 2 cases, upper limb arteries in one case, pulmonary artery in 2 cases and abdominal aorta in one case. Lower limb ischemia had occurred in one patient. Two patients (10%) had mixed vascular, arterial and venous involvement, confirming the multiple and ubiquitous nature of angio-Behçet. The treatment was based on colchicine, anticoagulants and corticosteroids in venous thromboses. Immunosuppressive therapy was started in 4 patients in front of the unusual site. In arterial cases, corticosteroids in combination with immunosuppressant were prescribed. Flattening of the aneurysm was indicated in 2 cases with simple operative follow-up. <h3>Conclusion:</h3> Behçet disease (BD) is very common in the Mediterranean basin, mainly affects the 30-year-old man. This systemic disease is characterized by oral aphthosis, genital ulcers and systemic involvement including occular, gastrointestinal, neurological, and vessels that make the severity of the disease. All types of vessels, regardless of size and seat, may be affected, with venous tropism. Our study has just supported current literature data regarding the extensive and recurrent venous thrombosis during MB. The seriousness of this venous involvement lies in the involvement of the cavernous veins and in pulmonary embolism. Arterial damage, such as thrombosis and/or aneurysm, is rare but maybe life-threatening. Early diagnosis, intensive and appropriate treatment, regular follow-up and the involvement of a multidisciplinary team including internists, vascular surgeons and radiologists are key to better management of patients with angio -Behçet. Our study illustrates the frequency and significance of vascular involvement in BD. <h3> Disclosure of Interests:</h3> None declared