Author/Editor     Kraut, Aleksandra
Title     Epidemiološke in klinične značilnosti Behcetove bolezni v Sloveniji
Translated title     Epidemiological and clinical features of Behcet disease in Slovenia
Type     članek
Source     Zdrav Vestn
Vol. and No.     Letnik 74, št. 10
Publication year     2005
Volume     str. 573-7
Language     slo
Abstract     Behcet 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, Cvclophosphamide 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 irnmunosuppressive 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.
Summary     Behcetova bolezen (BB) je kronična večsistemska vnetna bolezen neznanega vzroka. Najočitnejše so muko-kutane in očesne spremembe s pogosto hudo izgubo vida. Članek namerava prikazati prisotnost BB z očesno prizadetostjo pri bolnikih v Sloveniji. Metode. Retrospektivno smo analizirali popise in ambulantne kartone bolnikov z očesno BB, ki so se zdravili na Očesni kliniki med januarjem 1984 in decembrom 2004. Upoštevali smo mednarodno sprejeta merila za diagnozo bolezni. Pri rezultatih smo upoštevali spol, starost ob začetku uveitisa, očesne znake in zaplete na očeh, incidenca HLA B5 oziroma 51, sistemsko zdravljenje, zapleti zdravljenja in končna vidna ostrina. Rezultati. V opazovanem obdobju je bilo zdravljenih 25 bolnikov zaradi očesne BB. 16 bolnikov je bilo moških, 9 je bilo žensk. Začetek uveitisa je nastopil med 16 in 47. letom, v povprečju pri 34,2 leta. Vsi bolniki so imeli ponavljajoči se obojestranski panuveitis ali vaskulitis mrežnice. 15 bolnikov je bilo HLA B5 oziroma 51 pozitivnih, 5 je bilo negativnih in 5 bolnikov ni bilo testiranih. Vsi bolniki so poleg lokalne prejemali sistemsko zdravljenje: kortikosteroide 25 bolnikov azatioprin 13, ciklosporin A 11, ciklofosfamid 6, klorambucil 1 in kolhicin 1 bolnik. Končna vidna ostrina je bila: dojem svetlobe negativen pri 2 očeh, < 0,2 pri 13 očeh, med 0,2 do 0,6 pri 12 očeh in več kot 0,6 pri 23 očeh. Zaključki. BB z očesno prizadetostjo je v Sloveniji redka bolezen. Kljub klasičnem imunosupresivnem zdravljenju je pri 15 očeh (30%) prišlo do hude izgube vida. Za ohranitev vida je pomembno zgodnje odkritje očesne prizadetosti in ustrezno zdravljenje, ki postaja tudi bolj učinkovito in ciljano.
Descriptors     BEHCET'S SYNDROME