Characteristics of optic neuropathy in Behçet disease

Abstract

Objective: We present the clinical profile, features, and neuroimaging findings of 25 patients with Behçet disease (BD), and optic neuropathy (ON), which has been rarely reported in BD.

Methods: Data from 5 university hospitals were retrospectively reviewed, and patients with BD and ON were evaluated. There were 2 groups: (1) those already diagnosed with BD when ON developed (BD→ON group) and (2) those diagnosed with BD during the evaluation of ON (ON→BD group).

Results: There were 25 BD patients with ON (13 males). Among these, 13 had ON→BD, and 12 had BD→ON. Seventeen patients had unilateral ON, and 7 patients had recurrent ON. BD→ON patients were older. Disc edema was seen more in ON→BD than in BD→ON patients (10 vs 3). Fourteen patients also had uveitis, 7 with BD→ON and 7 with ON→BD. There was other neurologic involvement in 8 patients; in the BD→ON group, 4/4 had MS-like disease, in the ON→BD group, 3 had typical parenchymal BD, and 1 had MS-like disease. Twenty of 21 patients received immunosuppressive medications, corticosteroids, or both. Prognosis was favorable in most: vision improved in 20 patients, more often in those receiving combined therapies.

Conclusion: BD may be diagnosed earlier if it is considered and investigated during the assessment of ON, particularly in high-risk regions. Prognosis of ON related to BD seems to be favorable. Immunosuppressants should be given along with corticosteroids. MS-like presentations should also be kept in mind in patients with BD and ON.

Figure. Color fundus photographs

Color fundus photographs of a 35-year-old woman with newly diagnosed Behçet disease showing slight haze in vitreous, prominent disc edema, cotton wool spots, and some retinal hemorrhages in the right (A) and left (B) eyes. Venous phase of the fluorescein angiogram depicting marked disc leakage, scattered retinal capillary leakage, and hypofluorescent areas corresponding to the cotton wool spots and retinal hemorrhages in the right (C) and left (D) eyes. Optical coherence tomography of the macula exhibiting subfoveal serous neurosensory retinal detachment and thickened retina in the right (E) and left (F) eyes. The retinal nerve fiber layer was thickened in the right (G) and left (H) eyes. Humphrey 30.2 visual field test showing mainly peripheral visual field defects with visual field constriction in the right (I) and left (J) eyes.