BACKGROUND: Behçet disease (BD) is a chronic, inflammatory, multisystemic vasculitic disorder with a wide spectrum of clinical presentations. The highest prevalence is seen in Turkey. Specific diagnostic tools are yet to be discovered; thus, the diagnosis relies on physicians being acquainted with the symptoms and signs of the disease. OBJECTIVE: To investigate the epidemiologic characteristics of BD and to emphasize the typical clinical and laboratory characteristics. METHODS: This was a retrospective analysis of all the BD patients attending the Ankara Numune Education and Research Hospital throughout the years 2001-4. Diagnosis of BD was made according to the International Study Group criteria. A total of 213 patients were evaluated with respect to family history, clinical features, pathergy test, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), antistreptolysin O, and ferritin levels. When assessing disease activity, the active clinical manifestations on the day of the examination were taken into account. Correlations were analyzed between sex and age distribution, age of onset, disease duration, and family history; and between family history and age of onset, pathergy, clinical manifestations, and laboratory parameters. Correlations were also evaluated between pathergy positivity and clinical manifestations, and laboratory parameters. Correlations between activity scores and age of onset, duration, sex, family history, and laboratory data were also analyzed. RESULTS: The female : male ratio was 1.04, and the mean age of onset was 27 years. Family history did not affect age of onset or disease severity. Men presented with more active disease, and there was a weak but positive correlation between disease activity and CRP. No correlation was observed between duration and age of disease onset. The most common clinical presentations were oral aphthous lesions, genital ulcers, and skin lesions. Men more commonly presented with papulopustular lesions, pathergy positivity, and vascular, eye, and renal involvement, and women presented with arthritis/arthralgia more commonly than men. Vascular lesions, ESR, and CRP showed significant relationships with pathergy reaction. Eye involvement was not affected by age of onset. CONCLUSIONS: We believe our results indicate that the pathogenesis of BD is multifactorial. Hormonal factors seem to be of some influence, while genetic background and environmental factors seem to be the major contributors. Infections seem to be among the triggering environmental factors. Predisposing genes may affect the influence of environmental factors. Prevalence studies should be carried out periodically in those countries with a high prevalence of BD to keep up with the changing dynamics of the disease, which may also shed light on the as-yet unknown pathogenesis of BD.
BACKGROUND: Behçet disease (BD) is a chronic, inflammatory, multisystemic vasculitic disorder with a wide spectrum of clinical presentations. The highest prevalence is seen in Turkey. Specific diagnostic tools are yet to be discovered; thus, the diagnosis relies on physicians being acquainted with the symptoms and signs of the disease. OBJECTIVE: To investigate the epidemiologic characteristics of BD and to emphasize the typical clinical and laboratory characteristics. METHODS: This was a retrospective analysis of all the BD patients attending the Ankara Numune Education and Research Hospital throughout the years 2001-4. Diagnosis of BD was made according to the International Study Group criteria. A total of 213 patients were evaluated with respect to family history, clinical features, pathergy test, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), antistreptolysin O, and ferritin levels. When assessing disease activity, the active clinical manifestations on the day of the examination were taken into account. Correlations were analyzed between sex and age distribution, age of onset, disease duration, and family history; and between family history and age of onset, pathergy, clinical manifestations, and laboratory parameters. Correlations were also evaluated between pathergy positivity and clinical manifestations, and laboratory parameters. Correlations between activity scores and age of onset, duration, sex, family history, and laboratory data were also analyzed. RESULTS: The female : male ratio was 1.04, and the mean age of onset was 27 years. Family history did not affect age of onset or disease severity. Men presented with more active disease, and there was a weak but positive correlation between disease activity and CRP. No correlation was observed between duration and age of disease onset. The most common clinical presentations were oral aphthous lesions, genital ulcers, and skin lesions. Men more commonly presented with papulopustular lesions, pathergy positivity, and vascular, eye, and renal involvement, and women presented with arthritis/arthralgia more commonly than men. Vascular lesions, ESR, and CRP showed significant relationships with pathergy reaction. Eye involvement was not affected by age of onset. CONCLUSIONS: We believe our results indicate that the pathogenesis of BD is multifactorial. Hormonal factors seem to be of some influence, while genetic background and environmental factors seem to be the major contributors. Infections seem to be among the triggering environmental factors. Predisposing genes may affect the influence of environmental factors. Prevalence studies should be carried out periodically in those countries with a high prevalence of BD to keep up with the changing dynamics of the disease, which may also shed light on the as-yet unknown pathogenesis of BD.
Authors: A Altenburg; A Mahr; C Maldini; C E Kneifel; L Krause; I Kötter; T Stache; N G Bonitsis; C C Zouboulis Journal: Ophthalmologe Date: 2012-06 Impact factor: 1.059