Ocular Manifestations of Behçet’s Disease

Abstract

Behçet's disease (BD) is a multisystemic autoimmune inflammatory disorder characterized by oral aphthous lesions, genital ulcerations, iridocyclitis with hypopyon, and skin lesions. While ocular manifestations occur in nearly 50% of the patients with Behçet's disease, ocular involvement is the initial manifestation in only less than 20% of the patients. Ocular Behçet's disease clinically presents iridocyclitis with or without hypopyon, vitritis, retinitis, occlusive retinal vasculitis, and cystoid macular edema. However, anterior uveitis is usually the only initial ocular manifestation; the most common form is panuveitis. The usual course of the disease is characterized by recurrent inflammatory periods. Recurrent inflammatory attacks may result in irreversible damage and significant visual loss. Early and effective treatment is required to prevent ocular morbidity. Recent developments in the treatment of ocular Behçet's disease like biological agents are promising with a rapid effect and high remission rates.

Coexistence of Polyarteritis Nodosa of the Vulva and Retina in a Behçet's Disease Patient: A Case Report

“…Furthermore, ocular BD presents as diffuse capillary and venous inflammation with areas of retinal necrosis and major vascular occlusion [16,22]. The absence of anterior uveitis, vitritis, and retinal vasculitis in our patient, which would be common findings in BD, make it less likely as the cause of her findings [6].…”

“…Behçet's disease (BD) is a chronic, relapsing, autoimmune inflammatory disorder that involves different organ systems [5]. It can be difficult and challenging to diagnose BD because there are no single laboratory tests or pathognomonic findings to aid diagnosis [6]. The International Criteria for BD, known as the ICBD criteria, is the classification/diagnosis criteria of choice to help the diagnosis of BD [5].…”

“…Anterior uveitis (AU), also known as iridocyclitis, is the most common ocular manifestation in BD. AU is rarely isolated and is often accompanied by posterior segment involvement [6]. Posterior segment findings may include the presence of vitritis (hyalitis), macular edema in the retina caused by vascular leakage, foci of necrotizing retinitis, and retinal vasculitis (RV).…”

“…Posterior segment findings may include the presence of vitritis (hyalitis), macular edema in the retina caused by vascular leakage, foci of necrotizing retinitis, and retinal vasculitis (RV). RV is mainly venous and often occlusive, which is pathognomonic for BD [6]. The cutaneous manifestations of BD may have many forms including inflammatory pustules, pseudo-folliculitis, acneiform rash, erythema nodosum, pyoderma gangrenosumlike lesions, Sweet-syndrome-like lesions, superficial migratory thrombophlebitis, palpable purpuric lesions of necrotizing vasculitis, and suppurative panniculitis [7][8][9].…”

Case Report: Inflammatory orbital pseudotumor revealing Behçet's disease: an original observation

“…Eye involvement is common, occurs in 28-50% of cases, and is dominated by uveitis. 1,2 Clinical case A 28-year-old Tunisian male patient was admitted for right orbital protrusion and edema (Figure 1) with local pain and intense helmet headache lasting for 15 days before admission.…”