P206 Uniqueness of venous thrombosis in Behçet's syndrome: a case series of 62 patients
Authors: Chadli Sarra , Khibri Hajar , Benachour Rim , Fari Safaa Firdaous , Moatassim Naima , Ammouri Wafaa , Maamar Mouna , Harmouche Hicham , Adnaoui Mohammed , Mezalek Zoubida Tazi 
Published: April 2023

Background/Aims

Behçet’s syndrome (BS) is a complex inflammatory disorder frequently observed in the so-called “Silk Road”. Venous thrombosis (VT) is common in BS and known to be a predictive factor of future severe manifestations of the disease. Our work aims to describe the clinical and imaging characteristics of VT in BS, in order to shed the light on its specific features for early diagnosis and management of these patients.

Methods

We conducted a retrospective, descriptive and monocentric study, including 200 BS patients admitted to our internal medicine department between 2014 and 2021. All patients fulfilled the International Study Group criteria for Behçet’s disease.

Results

VT was found in 62 patients (31%). The mean age was 29 ± 10.8 (18-61) and the sex ratio (M:F) was 7.85:1. Family history of BS was documented in 5% of the cases. VT was either the revealing manifestation of the syndrome (56%) or occurred after a median of 3.5 years (1; 6.5) following the disease onset. Four patients had superficial venous thrombosis (SVT) mostly affecting the superficial femoral vein (98%). All patients had deep vein thrombosis (DVT), visualised in the lower extremities (58%), superior vena cava (21%), inferior vena cava (16%), cerebral veins (13%), and suprahepatic veins (5%). Atypical sites of DVT occurred in the renal (n = 1), axillary (n = 1), central retinal (n = 1), and dorsal penile (n = 1) veins. DVT was observed in one vein (55%), two veins (27%), or multiple (3 to 7) veins (16%). Thrombi sizes ranged from 10 to 40 mm, being willingly extended (55%) and strongly adherent to the vessel walls (95%). Concomitant arterial lesions were found in 34% of the patients, mainly in the pulmonary arteries (57%). They consisted of aneurysms (52%), thrombosis (43%), stenosis (17%), and pseudoaneurysms (9%). Cardiac lesions were also observed (11%), including right intracardiac thrombosis (n = 4), coronary artery thrombosis (n = 2), pericarditis (n = 1), and myocarditis (n = 1). The other extravascular involvements associated were mucocutaneous (100%), ocular (42%), neurological (29%), articular (24%), and digestive (23%). Fever was reported in 4 cases. All patients were treated with colchicine and glucocorticoids. Immunosuppressive therapy was prescribed in 75% of the cases: cyclophosphamide (n = 31), azathioprine (n = 12), and TNF alpha inhibitor (n = 3). Curative anticoagulation was also administered (98%). Two patients underwent surgery: thrombectomy (n = 1) and embolectomy (n = 1). Relapse occurred in 27% of the patients. No death was recorded.

Conclusion

VT is widely considered to be the hallmark of BS as well as a major risk factor for future severe vascular and cardiac involvements. Due to its inflammatory pathogenesis, it has specific clinical and imaging features. Better knowledge of these characteristics could help early establish the diagnosis of BS, thus improving patient outcomes.

Disclosure

S. Chadli: None. H. Khibri: None. R. Benachour: None. S. Firdaous Fari: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.