Fig. 12.15 Vascular wall staining on the superior arcuate vein and capillary dye leakage on fluorescein angiography

Fig. 12.16 Fluorescein angiography shows cystoid macular edema in a patient with Behçet’s disease

Fig. 12.17 Vascular remodeling on fluorescein angiography: retinal telangiectasias, collateral venous channels, and dilated capillary nets, as a result of capillary nonperfusion

Fig. 12.18 Dye leakage from disc neovascularization on fluorescein angiography

Fig. 12.22 (a) Cystoid macular edema, disc hyperfluorescence and retinal vasculitis on fluorescein angiography. (b) Cystoid macular edema on optical coherence tomography

medication, iris atrophy, and secondary glaucoma may develop. In the very late stages of the disease, an atrophic retina, optic atrophy, sheathed vessels, chorioretinal scars, and/or proliferative vitreoretinopathy (Fig. 12.26) are often observed. Neovascular glaucoma may occur in as many as 12% of patients and often results in phthisis bulbi [41].

Histopathology

Histopathological studies on eyes with Behçet’s disease showed a non-granulomatous uveitis and necrotizing, leukocytoclastic, and obliterative vasculitis, which affect arteries and veins of all sizes [42–44]. Retinal detachment, and diffuse or focal infiltration of the choroid with inflammatory cells were detected. Immunoglobulin and complement deposition in choroidal veins have been reported [45]. During acute inflammation, there is severe vasculitis with marked infiltration of leukocytes in and around blood vessels. Retinal vessels have thickened basement membranes with swollen endothelial cells, which can lead to thrombus formation and vascular obliteration [46]. In addition, the iris,

Fig. 12.24 Episcleritis

Fig. 12.25 Cataract and posterior synechia

Fig. 12.26 End-stage Behçet’s disease

ciliary body, and choroid show diffuse infiltration with neutrophils. In the late stages, there is proliferation of collagen fibers, thickening of the choroid, formation of cyclitic membrane, and sometimes hypotonia and phthisis bulbi. Lymphocytic and plasma cell infiltration occurs during remission. Of all ocular tissues, the retina suffers the most damage.

Prognosis of Ocular Disease

Ocular and central nervous system involvements are the main prognostic determinants in Behçet’s disease [19]. The ocular inflammatory episodes in Behçet’s disease are characteristically associated with a sudden severe onset of visual loss that may gradually improve with remission. The severity and number of repeated inflammatory attacks involving the posterior segment determine the extent of permanent structural changes and the resultant rate of irreversible visual loss [15]. Therefore, anterior uveitis alone carries the best prognosis in ocular involvement. A study from 1970 reported that 73% of patients with ocular disease developed permanent loss of vision within an average time of 3.5 years [47]. Similarly, another study from 1986 reported that 74% of treated patients lost useful vision 6–10 years after the onset of symptoms [48]. However, increased awareness of the disease, a more aggressive treatment approach, and the availability of several immunosuppressive agents seem to have improved the prognosis of Behçet’s disease in the last decade [15, 48].

The clinical course of the disease shows individual variability even in the same family [49]. Nevertheless, male patients in general have a higher risk of eye involvement, younger age at disease onset, more severe disease, and a higher risk of visual loss compared to female patients [15, 19]. In Japan, more than 50% of male patients lose visual acuity to less than 0.1 in 5 years, but this is the case in only 10% of female patients [11]. Consequently, Behçet’s disease is the cause of blindness in about 12% of acquired blindness in adults in Japan.

In the majority of studies, early age at onset is found to be associated with a more severe disease