Behçet's Uveitis

Abstract

Behçet's disease is a multisystem inflammatory disorder that is most common in countries along the ancient "Silk Road". The eye is the most commonly involved vital organ in Behçet's patients and the typical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Uveitis is the initial manifestation of the disease in 10-15% of the patients. Anterior uveitis is always nongranulomatous. Diffuse vitritis, retinal infiltrates, sheathing of predominantly retinal veins, and occlusive vasculitis are the typical signs of posterior segment inflammation. Spontaneous resolution of acute inflammatory signs is a diagnostic feature. Fundus fluorescein angiography is the gold standard in monitoring inflammatory activity. Laser flare photometry is a useful noninvasive tool since flare readings correlate with fluorescein angiographic leakage. The most common complications are cataract, maculopathy, and optic atrophy. Male patients have a more severe disease course and worse visual prognosis. Immunomodulatory therapy is indicated in all patients with posterior segment involvement. Corticosteroids combined with azathioprine and/or cyclosporine is used initially. Biologic agents, including interferon alfa and infliximab, are used in resistant cases. Visual prognosis has improved in recent years with an earlier and more aggressive use of immunomodulatory therapy and the use of biologic agents in resistant cases.

Keywords: Behçet's Disease; Immunomodulatory Treatment; Retinal Vasculitis; Uveitis.

Figure 1a

(a) A 27 year-old pregnant woman with Behçet's disease presented with a hypopyon panuveitis;

Figure 1b

(b)with multifocal retinal infiltrates;

Figure 1c

(c)Spontaneous resolution of the retinal infiltrates and accumulation of precipitates on the surface of the inferior peripheral retina were noted one week later

Figure 2a

(a)Color fundus photograph of the left eye of a patient with Behçet's disease shows retinal hemorrhages at the posterior pole and at the superior temporal quadrant following an episode of superior temporal branch retinal vein occlusion

Figure 2b

(b) fluorescein angiography shows staining of the optic disc as well as inflamed retinal vessels and hypofluorescence due to retinal hemorrhages and nonperfusion of the retinal vasculature at the involved quadrant

Figure 3

Color photograph shows end-stage fundus picture in the left eye of a patient with Behçet's disease. Please note optic atrophy, gliotic vascular sheathing, ghost vessels, and diffuse retinal atrophy with pigmentation resembling retinitis pigmentosa