Behçet’s disease

Abstract

Abstract Purpose Patients with Behçet’s disease have recurrent inflammatory attacks in all organ systems involved. Ocular involvement is characterized by nongranulomatous panuveitis and retinal vasculitis with a relapsing and remitting course. The demographic and clinical features of Behçet's uveitis and treatment will be discussed. Methods Literature review Results The onset of uveitis is typically in the third or fourth decade of life. Uveitis is more frequent and its course is more severe in male patients. The typical findings of uveitis attacks include anterior chamber cells with or without a hypopyon formation, diffuse vitreous haze, occlusive periphlebitis, retinal infiltrates, and inflammation of the optic disc. In severe attacks, diffuse retinal edema and even a serous retinal detachment may develop. Macular edema is the most common complication. Recurrent attacks lead to permanent loss of useful vision due to optic atrophy, maculopathy, and diffuse atrophy and gliosis of the retina. Fluorescein angiography is the gold standard in monitoring disease activity. Anterior chamber flare measured by laser flare photometry correlates with fluorescein leakage in the posterior segment. Management of Behçet uveitis is based on the rapid control of intraocular inflammation and prevention of recurrences. Corticosteroids are generally used for the treatment of acute attacks. However, patients with posterior segment involvement require long‐term immunomodulatory treatment in order to prevent recurrences. Both azathioprine and cyclosporine have been shown to be effective in controlled trials. Interferon alfa or infliximab is used in resistant cases. Conclusion Behçets uveitis is resistant to conventional treatment in around 20% of patients. Visual prognosis has improved in recent years with the use of biologic agents