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Background Behcet’s syndrome (BS) is an auto-inflammatory vasculitis, most common in countries along the ancient Silk Road. Classification of BS most often is done with the International Study Group criteria (ISG criteria)1. ISG criteria positivity for BS is reached when a patient has recurrent oral ulceration and any two of the following symptoms: recurrent genital ulceration, uveitis, skin lesions and pathergy positivity. However, many other manifestations are reported1. Differences in clinical presentation can complicate classification of the diagnosis, especially in areas where the disease is low in prevalence. Thus, some patients are classified as “probable BS”. In some of these cases patients developed additional symptoms over time and met the ISG criteria in a later stage. Objectives To describe characteristics of patients presenting with a probable diagnosis of BS in Amsterdam and New York and to study if patients who eventually met the ISG criteria differ from those who did not. Methods We included consecutive patients classified as possible BS to our outpatient clinics in New York and Amsterdam. Patients fulfilling ISG criteria at enrollment were excluded, as well as patients in whom an alternative diagnosis was made at enrollment. Baseline data were evaluated retrospectively and patients were divided into two groups: those developing ISG positive (ISG +or “true”) BS during follow up and those who did not meet ISG criteria after follow-up (ISG-). Turkey, Asia, Middle and Far Eastern countries, Arabic countries and Northern Africa were considered endemic areas; Italian, Greek, Hispanic, Portugese, African-American and Caucasian patients were considered not from endemic areas. Statistical analysis was performed using SPSS, with Chi-square tests or Fisher’s exact tests for categorical data and independent sample t-tests for numerical data. Results 189 patients were included, of whom 20 were from Amsterdam. During follow up, 71 patients (37.6%) could be classified as “true” Behcet’s syndrome after a mean period of 9.4 years (±8.3 years) after onset of symptoms. Age, gender, ethnicity, duration of symptoms at enrollment, duration of follow up as well as RAPID3 and almost all clinical manifestations at baseline were comparable for both groups. Labial ulcers and skin manifestations at enrollment were more frequently reported. Genital ulcers as a group was not significantly associated with developing “true” Behcets, nor were specific skin manifestations such as erythema nodosum. We also considered HLA-B*51, pathergy, erythrocyte sedimentation rate and C-reactive protein, but due to a large amount of missing data, we were unable to draw any significant conclusions for these variables. Figure 1 Baseline data from all patients who were classified as probable Behcet’s syndrome at enrollment Conclusions About a third of patients classified as probable Behcet’s syndrome at enrollment develop new manifestations over time. Thus, they can be reclassified as ISG positive (“true”) BS. Based on our data, presence of skin manifestations and labial ulcers at enrollment was significantly higher in the group eventually developing a ISG criteria positive BS. Reference [1] Criteria for diagnosis of Behcet’s disease. International Study Group for Behcet’s Disease. Lancet1990;335(8697):1078–80. Disclosure of Interest: F. Kerstens Grant/research support from: Stichting Jan van Breemen Grant, F. Turkstra: None declared, C. Swearingen: None declared, Y. Yazici: None declared |
