• Z Tazi Mezalek, H Khibri, S El Fari, S Chadli, W Ammouri, M Maamar, H Harmouche, and M Adnaoui.
• Service médecine interne, centre hospitalo-universitaire Ibn Sina, Rabat, Maroc; Service hématologie clinique, centre hospitalo-universitaire Ibn Sina, Rabat, Maroc; Université Mohammed V, faculté médecine et pharmacie, Rabat, Maroc. Electronic address: z.tazimezalek@gmail.com.
• Rev Med Interne. 2023 Feb 1; 44 (2): 727872-78.

AbstractBehçet disease is a multi-systemic complex vasculitis with unknown etiology characterized by different clinical involvements, including mucocutaneous, ocular, vascular, articular, neurological and gastrointestinal manifestations. Growing evidence supports that different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, the vascular phenotype identifies a specific group of patients who suffer from recurrent inflammatory thrombosis and arterial involvement. Vascular disease develops in up to 40% with a definite male preponderance and is usually an early manifestation. It is one of the main causes of death in Behçet's disease. Venous involvement is significantly more common than arterial disease and lower extremity deep vein thrombosis is its most frequent manifestation. Arterial disease involves mostly pulmonary arteries and aorta and manifests mainly in the form of aneurysms. Glucocorticoids and immunosuppressant's are the recommended first-line treatments in vasculo-Behçet. Furthermore, randomized controlled trials are still needed to assess the role of adding anticoagulation to current standard therapy in venous thrombosis in Behçet's disease and to assess the role of anti-TNF alpha therapy in vasculo-Behçet.Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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