Background: Adamantiadis-Behçet's disease is a chronically progressing multisystemic disorder. The underlying disease mechanism is an obliterative vasculitis of unknown etiology. Main clinical symptoms are oral and genital aphthous ulcers and intraocular inflammations. Additionally, cutaneous, rheumatoid, neural, gastrointestinal, or cardiovascular manifestations may be observed. 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 disease.
Patients and methods: In a retrospective fashion 196 patients of the Department of Ophthalmology of the University of Erlangen-Nürnberg between 1988 and 1998 with retinal vasculitis seen by fluorescence angiography were evaluated according to the diagnostic criteria of the "Behçet's Disease Research Committee of Japan".
Results: Among 196 patients there were 12 patients with Adamantiadis-Behçet's disease. Apart from retinal vasculitis, angiographic features included capillary dropout in 64% (9/14), swelling of the optic disc in 79% (11/14) and irregularly delayed areolar filling of choriocapillaris in the early phase of fluorescence angiography in 43% (6/14) of eyes. There was no statistically 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 Adamantiadis-Behçet's disease presented a delayed choroidal filling in fluorescence angiography as a sign of choroidal involvement of occlusive vasculitis. We observed leakage of fluorescein from the optic disc, which could be due to a secondary inflammation of the ciliary circulation. Inflammatory involvement predominantly of choroidal vessels, as visualized in fluorescence angiography, may be a diagnostic lead in Adamantiadis-Behçet's disease.