Ocular Involvement of Behçet’s Syndrome: a Comprehensive Review

Abstract

Behçet's syndrome (BS) is a vasculitis involving several organ systems including the eyes. Ocular involvement is one of the most disabling complications of BS, causing loss of vision that may progress to blindness if left untreated. The typical form of ocular involvement is a relapsing and remitting panuveitis and retinal vasculitis. Initial attacks may spontaneously improve and subsequently disappear in a few weeks but tend to recur if left untreated. Destructive and recurrent attacks, especially with posterior segment and retina involvement, may cause irreversible ocular structural changes and permanent damage in sensory retina, resulting in loss of vision. The risk of irreversible damage to ocular tissue which may result in loss of vision warrants early and intensive treatment especially in patients at high risk such as young men who tend to follow an aggressive disease course. The management strategy involves rapid suppression of inflammation during the attacks and prevention of recurrent attacks. Local and systemic measures including immunosuppressives, corticosteroids, and biologic agents are used for this purpose. Surgery may be required in selected cases. The prognosis of eye involvement has greatly improved over the last decades with the effective use of immunosuppressives.

Treating the Different Phenotypes of Behçet's Syndrome

“…In a first post-mortem study on a BS patient with parenchymal involvement, a cell infiltration was found around the central retinal artery within the optic nerve (86). Eye involvement is present in around half of BS patients, with a higher prevalence in males, and a lower prevalence among elderly (87). Ocular involvement is one of the most disabling complication in BS (87).…”

“…Eye involvement is present in around half of BS patients, with a higher prevalence in males, and a lower prevalence among elderly (87). Ocular involvement is one of the most disabling complication in BS (87). In a retrospective observational study on 295 BS patients, a significant association between posterior uveitis and parenchymal CNS involvement was reported (34).…”

Uveitis bei Behçet-Syndrom in Deutschland – Prognose unter aktueller Therapie

“…Die Augenmanifestationen treten meistens innerhalb der ersten 5 Jahre nach Krankheitsbeginn auf. Eine beidseitige Augenbeteiligung ist häufig und wird bei 75-80 % der Patienten beobachtet [14]. Die Uveitis ist Erstsymptom der Erkrankung bei 10-15 % der Patienten, weswegen es wichtig ist, die Augenbeteiligung bei Behçet-Syndrom auch bei noch fehlenden Systemmanifestationen zu erkennen [15].…”

Refractory Ocular Behçet’s Disease; Outcome of Low Dose Infliximab

“…Even with intensive immunosuppressive therapy, however, ocular inflammation may relapse in about 70% of patients, resulting in permanent loss of vision. The prognosis of OBD has improved significantly over the last decades with the use of immunosuppressives [6,[20][21][22][23][24][25][26]. Fair results have been described with pulse cyclophosphamide and AZA [27].…”