Epidemiological and clinical features of Behçet disease in Slovenia
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.
International Study Group for Behçet disease. Criteria for diagnosis of Behçet disease. Lancet 1990; 335: 1078–80.
Behçet’s Disease. In: Nussenblatt RB, Whitcup SM, Palestine AG. Uveitis, fundamentals and clinical practice. St. Louis: Mosby; 1996.
Verity DH, Wallace GR, Vaughan RW, Stanford MR. Behçet’s disease: from Hippocrates to the third millenium. Br J Ophthalmol 2003; 87: 1175–83.
Azizlerli G, Köse AA, Sarica R, Gul A, Tutkum IT, Kulac M, et al. Prevalence of Behçet disease in Istambul, Turkey. Int Dermatol 2003; 42: 803–6.
Vidan-Jeras B. Vrednotenje uporabnosti statističnih metod populacijske genetike pri določanju polimorfizma genov HLA I. razreda v slovenski populaciji [magistrsko delo]. Ljubljania: Univerza; 2002.
Tugal-Tutkun I, Onal S, Altan-Yaycioglu A, Altunbas HH, Urgancioglu M. Uveitis in Behçet disease: An analysis of 880 patients. Am J Ophthalmol 2004; 138: 373–80.
Probst K, Fijnheer R, Rothova A. Endothelial cell activation and hypercoagulability in ocular Behçet’s disease. Am J Ophthalmol 2004; 137: 850–7.
Muhaya M, Lightman S, Ikeda E, Mochizuki M, Shaer B, McCluskey P, et al. Behçet disease in Japan and in Great Britain: a comparative study. Ocul Immunol Inflamm 2000; 8: 141–8.
Yazici H, Yurdakul S, Hamuryudan V. Behçet’s syndrome. Curr Opin Rheumatol 1999; 11: 53–7.
Özyazgan Y, Yurdakul S, Yazici H, Tüzün B, Işçimen A, Tüzün Y, et al. Low dose Cyclosporin A versus pulsed cyclophosphamide in Behçet’s syndrome: a single masked trial. Br J Ophthalmol 1992; 76: 241–3.
Ozdal PC, Ortac S, Taskintuna I, Firat E. Long-term therapy with low dose cyclosporin A in ocular Behçet’s disease. Doc Ophthalmol 2002; 105: 301–12.
Durand JM. Interferon-α2b for refractory ocular Behçet’s disease. Lancet 1994; 344: 1994.
Kötter I, Zierhut M, Eckstein AK, Vonthein R, Ness T, Günaydin I, et al. Human recombinant interferon alfa-2a for treatment Behçet’s disease with sight threatening posterior or panuveitis. Br J Ophthalmol 2003; 87: 423–31.
Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN. Effect of Infliximab in sight-treatening panuveitis in Behçet’s disease. Lancet 2001; 358: 295–6.
Joseph A, Raj D, Dua HS, Powell PT, Lanyon PC, Powel RJ, et al. Infliximab in the treatment of refractory posterior uveitis. Ophthalmology 2003; 110: 1449–53.
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