Old and New Challenges in Uveitis Associated with Behçet’s Disease

Abstract

Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD.

Novel approach to Behçet's disease in the era of biologic agents

“…On the contrary IL-12/23 inhibitor (ustekinumab) and JAK inhibitors in which demonstrate early promising result in controlling noninfectious uveitis, are still too early to conclude their efficacy for long-term anti-inflammatory control outcome [64 ▪ ,65]. Alternatively, rituximab can be used in ocular Behçet's disease but studies suggest that it was not better than older conventional therapy [66,67].…”

Ocular lesions in Behçet’s disease

“…Recurrent anterior uveitis may lead to glaucoma. Elevated intraocular pressure occurs as a result of closure of the iridocorneal angle caused by anterior synechiae or as a result of inflammation [17]. However, posterior segment involvement is more common in BD (50-93% of cases) and may include vitritis, retinal vasculitis, retinal vascular occlusion, and cystoid macular oedema [16].…”

“…Another important examination complementary to angiography is optical coherence tomography that enables imaging of pathologies within the macula flava (macular oedema, retinal cysts, retinal detachment, atrophy of fovea, etc.) [17]. Recent studies are looking for methods for early diagnosis of ocular lesions in BD.…”

Management of Non-Infectious Uveitis, a Selection of Topical Items Updating

“…Among patients with Behcet’s uveitis, 90% have posterior uveitis or panuveitis and 78% have bilateral involvement; furthermore, 89% have retinal vasculitis and 44% macular edema [ 23 , 24 ], which can lead to vision loss (with blindness in 13% to 21% of cases in series published in the last century) [ 23 , 24 , 25 ]. In this Special Issue, Gueudry et al provided current data on how innovations in clinical evaluation, investigations, and treatments were able to improve the prognosis of uveitis associated with BD [ 26 ]. According to the 2018 EULAR recommendations for the management of BD, posterior segment involvement warrants systemic glucocorticoid therapy in combination with azathioprine or ciclosporin [ 27 ].…”