• A-C Desbois, C Terrada, P Cacoub, B Bodaghi, and D Saadoun.
• Sorbonne universités, UPMC université Paris 06, UMR 7211, et DHU i2B-inflammation-immunopathologie-biothérapie, 75005 Paris, France; Inserm, UMR_S 959, 75013 Paris, France; CNRS, FRE3632, 75005 Paris, France; Département de médecine interne et d'immunologie clinique, université Pierre-et-Marie-Curie, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France; Centre national de référence des maladies auto-immunes systémiques rares, centre national de référence des maladies auto-inflammatoires et de l'amylose, France.
• Rev Med Interne. 2018 Sep 1; 39 (9): 738-745.

AbstractOcular disease in Behçet's disease is frequent and may be associated with a poor functional prognosis. Uveitis is the most common ocular manifestation in Behçet's disease and represents a diagnostic criterion of the disease. The ocular involvement is inaugural of the disease in 20% of the cases or may develop 2 to 3 years after the beginning of the extraocular signs. The risk of blindness at 5 years is in the order of 15 to 25%, mainly due to macular involvement or retinal vasculitis. Uveitis may be anterior, intermediate, posterior or panuveitis. Anterior uveitis is rarely isolated and is frequently accompanied by posterior involvement. Anterior uveitis is always non granulomatous, sometimes associated with hypopion. Posterior involvement may include the presence of hyalitis, retinal vasculitis, mainly venous and often occlusive, macular edema, and/or foci of necrotizing retinitis. Behçet's disease is a chronic disorder, characterized by a relapsing and remitting course. Male patients with younger age at onset and severe lesions at presentation are at higher risk of severe visual loss over time. The main goals in the management of patients with Behçet's disease-associated uveitis are rapid suppression of intraocular inflammation, preservation of vision, and prevention of recurrences. The treatment is based on the use of systemic glucocorticosteroids and immunosuppressive agents. Posterior segment involvement requires the use of corticosteroids and immunosuppressants, primarily azathioprine. This treatment does not appear to be sufficient for severe uveitis with reduced visual acuity or retinal vasculitis that requires anti-TNF α or interferon α. Therapeutic strategies such as TNF-alpha blockers have dramatically improved the visual prognosis of patients with intraocular inflammation related to this chronic and potentially blinding condition.Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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