Purpose
To investigate the mechanisms involved in the development of neovascularization of the optic disc (NVD) in Behçet's disease
and to evaluate the effects of medical and laser treatments.
Methods
This is a retrospective study of 26 consecutive Behçet's patients (38 eyes) with NVD who presented between 1990 and 2004 at
our university hospital. Information on age at presentation, sex, disease duration, laterality of NVD, ocular findings, fluorescein
angiographic findings, visual acuity, medical treatment, laser photocoagulation, surgical procedures, and the follow-up period
was collected. Eyes with diffuse capillary leakage on fluorescein angiography were defined as having inflammation-induced
NVD, and eyes with extensive retinal capillary nonperfusion were defined as having ischemia-induced NVD.
Results
Eighteen patients were male, and eight were female. Mean age at presentation was 25.4 ± 4.9 years. Median disease duration
was 5.5 months. Median follow-up was 24 months. Twelve patients had bilateral NVD; 14 had unilateral NVD. Inflammation-induced
NVD was seen in 87% of the eyes, and ischemia-induced NVD in 13%. Initial treatment with high-dose corticosteroids combined
with conventional immunosuppressive agents was effective in 45% of the eyes with inflammation-induced NVD. Retinal laser photocoagulation
was effective in three of five eyes with ischemia-induced NVD. Treatment with interferon α-2a resulted in resolution of NVD
in all seven patients who received this agent for inflammation- or ischemia-induced persistent NVD. Final visual acuity was
less than 0.1 in 24% of the eyes.
Conclusions
Inflammatory mechanisms seem to predominate in the pathogenesis of NVD in Behçet's disease. The results of this study suggest
that the response to intensive anti-inflammatory and conventional immunosuppressive treatment is not satisfactory; retinal
laser photocoagulation may be ineffective even in eyes with retinal ischemia, but interferon α-2a seems to be effective for
the treatment of Behçet's patients with NVD. Jpn J Ophthalmol 2006;50:256–265 © Japanese Ophthalmological Society 2006
Key words Behçet's disease - inflammation-induced neovascularization - interferon α-2a - ischemia-induced neovascularization - neovascularization of the optic disk
Presented at the XI International Conference on Behçet's Disease, 27–31 October 2004, Antalya, Turkey