and effectiveness in uveitic glaucoma associated with Behçet’s disease [98, 99].

Vitrectomy

Vitreoretinal surgery is aimed at intense vitreous condensation, non-resolving vitreous hemorrhage, persistent cystoid macular edema, epiretinal membrane, and tractional retinal detachment. Removal of mechanical and humoral factors in vitreous gel by vitrectomy may enable better control of inflammation with a decreased number, severity and duration of uveitis attacks, and better diffusion of drugs to posterior segment, thus better visual acuity [100, 101]. In addition, relieving ciliary traction by vitrectomy may also prevent the development of hypotony and phthisis bulbi [102].

Controversies and Perspectives

The advances in research in the field of immunology and genetics in the last decades enabled a better insight into the etiopathogenesis of Behçet’s disease. However, until the exact disease mechanism is found, the management is bound to be symptomatic rather than curative. Until then, new immunomodulatory/immunosuppressant agents with less systemic side effects may aid the prognosis of resistant cases. Most cases with Behçet’s disease require more than one medication to suppress inflammation and reduce the frequency of attacks. With the absence of a standard treatment protocol and large randomized, controlled trials, the physician should evaluate each patient on an individual basis and decide the treatment accordingly. Corticosteroids are still the first choice of treatment in ocular Behçet’s patients to treat acute inflammation. Cyclosporin A and interferon may be added to the management as the second-line treatment if necessary. Drugs such as infliximab and azathioprine or other immunosuppressants may be preserved for the most severe cases. With the use of such medications, the prognosis of the disease has significantly improved compared to 20 years ago.

Pearls

•The major characteristic of Behçet’s disease is a systemic vasculitis with mucosa, skin, and eye being predominantly involved.

•Although Behçet’s disease may affect almost any system of the body with exacerbations and remissions of inflammation, the key clinical manifestations are recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions such as erythema nodosum, pseudofolliculitis, papulopustular lesions, and acneiform nodules.

•HLA-B51 has been the most closely associated risk factor for Behçet’s disease.

•There are no specific laboratory tests and pathognomonic findings in Behçet’s disease. Therefore, the diagnosis remains largely a clinical one. It should be kept in mind, however, that recurrent oral ulcers are reported in almost all patients with Behçet’s disease.

•Ocular involvement as anterior, intermediate, posterior, or panuveitis is common. The uveitis is chronic, recurrent, and non-granulomatous.

•Ankylosing spondylitis, Reiter’s syndrome, inflammatory bowel diseases, sarcoidosis, Vogt-Koyanagi-Harada syndrome, Eales’ disease, and infectious diseases such as syphilis and tuberculosis are among the most common differential diagnosis of ocular Behçet’s disease.

•The aim of the treatment in ocular Behçet’s disease is to achieve a rapid resolution of inflammation, to reduce frequency and severity of attacks, and to avoid complications. There is not a standard treatment protocol, and the extent and severity of the disease determine the treatment required.

•Corticosteroids, cyclosporin A, interferon-a, anti-tumor necrosis factors (infliximab), and azathioprine are the most commonly used medications for ocular inflammation.

•Laser photocoagulation should be performed in areas of capillary non-perfusion and retinal neovascularization. In the presence of disc neovascularization, panretinal photocoagulation should be performed to prevent complications such as vitreous hemorrhage and neovascular glaucoma.

•Although variable, the prognosis of ocular Behçet’s disease is poor in the majority of patients. Nevertheless, increased awareness of the disease, a more aggressive treatment approach, and the availability of several immunosuppressive/modulators seem to have improved the prognosis of Behçet’s disease in the last decade.

Acknowledgment Adapted from Atmaca-Sonmez P, Atmaca LS, Aydintug OT. Update on ocular Behcet’s disease. Expert Rev. Ophthalmol. 2(6), 957–980 (2007) with permission of Expert Reviews Ltd.

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