Epidemiological and clinical features of Behçet disease in Slovenia
Abstract
Background: Behçet disease (BD) is a chronic multisystemic inflammatory disease of unknown cause. Most prominent features are muco-cutaneous and ocular leasons with frequent profound visual loss. The aim is to report the presence of BD with ocular involvement in patients in Slovenia.
Methods: A retrospective analysis of charts and outpatients records of patients with BD treated at University Eye Clinic of Ljubljana between January 1984 and Decembre 2004. International diagnostic criteria for the disease were considered. Main outcome measures were: sex, age at onset of uveitis, ocular features and complications, incidence of HLA B5 or 51, systemic treatment, complications of treatment, and final visual acuity.
Results: In the observational period 25 patients with ocular BD were treated. 16 patients were male, 9 were female. Onset of uveitis ranged between 16–47 years, mean at 34.2 years. All patients had recurrent bilateral panuveitis or retinal vasculitis. 15 patients were HLA B5 or 51 positive, 5 were negative and 5 were not tested. Beside local therapy all patients were on systemic therapy: Corticosteroids 25 patients, Azathioprine 13, Cyclosporine A 11, Cyclophosphamide 6, Chlorambucil 1, and Colhicine 1 patient. Final visual acuity was: light perception negative in two eyes, < 0.2 in 13 eyes, from 0.2 to 0.6 in 12 eyes, and > 0.6 in 23 eyes.
Conclusions: BD with ocular involvement is a rare disease in Slovenia. In spite of classic immunosuppressive therapy, in 15 eyes (30%) profound visual loss was observed. To maintain visual acuity an recognition of ocular involvement and appropriate treatment is important, as therapy is becoming more effective and determined.
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References
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