Adamantiadis-Behcet's disease: Fluorescence angiography and choroidal malperfusion

Background: Adamantiadis-Behcet's disease:is a chronically progressing mult isystemic disorder. The underlying disease mechanism is an obliterative vas culitis of unknown etiology. Main clinical symptoms are oral and genital ap hthous ulcers and intraocular inflammations. Additionally, cutaneous, rheum atoid, neural, gastrointestinal, or cardiovascular manifestations may be ob served. Diagnosis is based on clinical features since currently no specific laboratory tests or pathognomonic histopathological features are available . Ocular changes may provide important diagnostic clues to the systemic dis ease. Patients and methods: In a retrospective fashion 196 patients of the Depart ment of Ophthalmology of the University of Erlangen-Nurnberg between 1988 a nd 1998 with retinal vasculitis seen by fluorescence angiography were evalu ated according to the diagnostic criteria of the ,,Behcet's Disease Researc h Committee of Japan". Results: Among 196 patients there were 12 patients with Adamantiadis-Behcet 's disease. Apart from retinal vasculitis, angiografic features included ca pillary dropout in 64% (9/14), swelling of the optic disc in 79% (11/14) an d irregularly delayed areolar filling of choriocapillaris in the early phas e of fluorescence angiography in 43% (6/14) of eyes. There was no statistic ally significant relationship between severity of the systemic disorder and the activity of the ocular disease. Conclusion: Apart from retinal vasculitis, 43% of eyes in patients with Ada mantiadis-Behcet's disease presented a delayed choroidal filling in fluores cence angiography as a sign of choroidal involvement of occlusive vasculiti s. We observed leakage of fluorescein from the optic disc, which could be d ue to a secon-dary inflammation of the ciliary circulation. Inflammatory in volvement predominantly of choroidal vessels, as visualized in fluorescence angiography, may be a diagnostic lead in Adamantiadis-Behcet's disease.