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Behçet's disease (BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and skin lesions. Prevalence of BD is highest in countries along the ancient silk road from the Mediterranean basin to East Asia. By comparison, the prevalence in North American and Northern European countries is low. Gastrointestinal manifestations of Behçet's disease are of particular importance as they are associated with significant morbidity and mortality. Although ileocecal involvement is most commonly described, BD may involve any segment of the intestinal tract as well as the various organs within the gastrointestinal system. Diagnosis is based on clinical criteria - there are no pathognomonic laboratory tests. Methods for monitoring disease activity on therapy are available but imperfect. Evidence-based treatment strategies are lacking. Different classes of medications have been successfully used for the treatment of intestinal BD which include 5-aminosalicylic acid, corticosteroids, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody therapy. Like inflammatory bowel disease, surgery is reserved for those who are resistant to medical therapy. A subset of patients have a poor disease course. Accurate methods to detect these patients and the optimal strategy for their treatment are not known at this time.

Background: Currently, there is one Behçet's disease (BD) specific self reporting questionnaire developed and published in the literature, The Leeds BD-quality of life (QoL). We conducted a cross-cultural adaptation and validation of the Arabic version of the Leeds BD-QoL METHODS: A cross-sectional study was conducted among 41 consecutive patients attending rheumatology clinics at the American University of Beirut Medical Center between June and December 2007. The BD-QoL questionnaire, the Katz Index of Activities of Daily Living (ADL) and the Lawton Instrumental Activities of Daily Living (IADL) questionnaires were co-administered during the same visit, and severity scores were calculated. Cross-cultural adaptation of BD-QoL was performed using forward and backward translations of the original questionnaire. Internal consistency and test-retest reliability of the final version were determined. Exploratory Factor Analysis (EFA) was used to assess the dimensionality of the scale items. External construct validity was examined by correlating Arabic BD-QoL with the severity score, ADL and IADL., Results: The 30 items of the adapted Arabic BD-QoL showed a high internal consistency (KR-20 coefficient 0.89) and test-retest reliability (Spearman's test 0.91). The convergence of all 30 items suggests that the 30-item adapted Arabic BD-QoL scale is unidimensional. BD-QoL did not correlate with any of the patients' demographics. Still, it was positively correlated with patient severity score (r 0.4, p 0.02), and IADL (but not ADL)., Conclusions: This cross-cultural adaptation has produced an Arabic BD-QoL questionnaire that is now available for use in clinical settings and in research studies, among Arabic speaking patients.

Background and Aims: Polymorphisms in the tumor necrosis factor (TNF) gene at the locations -308, -238, -863, -857 and -1031 have been studied in various ethnic groups for possible association with Behçet's disease. The aim of this meta-analysis is to examine the association between polymorphism in the TNF gene at the locations -308, -238, -863, -857 and -1031 and Behçet's disease., Methods: A literature review was performed using MEDLINE, EMBASE and the Cochrane Central Register of Controlled Trials for original studies published in English up to October 31, 2009 and that examined the association of the TNF-alpha promoter polymorphisms with Behçet's disease. All pooled odds ratios (OR) were derived from random-effects model with its 95% confidence intervals (CI). We assessed statistical heterogeneity among studies using Cochrane Q test and by calculating I(2). The Cochrane collaboration's software program, RevMan 5 was used to prepare and complete this review., Results: The literature search resulted in 13 studies. Ten studies met the included criteria and thus were selected. Overall, -1031C (OR = 1.35, 95% CI = 1.09-1.68), -238A (OR = 1.51, 95% CI = 1.12-2.04) and -857T (OR = 0.76, 95% CI = 0.58-0.98) had a significant association with Behcet's disease. The pooled estimates for the other polymorphisms were not statistically significantly associated with Behcet's disease; -308A and -863A., Conclusions: Behcet's disease was associated with the -1031C, -238A and the -857T promoter polymorphisms in various ethnic groups., (Copyright 2010 IMSS. Published by Elsevier Inc. All rights reserved.)

The aim of this study is to describe the clinical features of Lebanese patients with Behçet's disease (BD) followed up at a tertiary care center in Lebanon. A retrospective review of medical records of 90 patients who fulfilled the International Study Group (ISG) criteria for diagnosis was performed. The clinical characteristics and severity score were compared with those reported from other populations of Arab and of Turkish origin using the same diagnostic criteria, and a comparison over two decades for the Lebanese population was made. The male-to-female ratio was 2.9:1, the mean age at onset, 25.4 years, and mean age at diagnosis, 29.2 years. One hundred percent of the patients had mouth ulceration; 72.2%, genital ulceration; 59.1%, arthritis; 55.7%, papulopustular skin lesion; 53.9%, ocular disease; 36.8%, vascular disease; 29.5%, erythema nodosum; and 23.0%, neurologic disease. The characteristics of our patient population are similar to that reported from other countries in the region. However, we found a higher prevalence of vascular and neurologic disease.

Behçet's disease is a chronic multisystem inflammatory disease characterized by the predominance of Th1 cytokines. The human leukocyte antigen-B 51 gene is associated with the disease in several ethnic groups, but it is not certain whether it is the susceptibility gene or if it is in linkage disequilibrium with other genes. Recent studies of polymorphisms of neighboring, as well as distant, genes to human leukocyte antigen-B 51 are helpful in elucidating the pathogenesis of the disease and could have therapeutic implications.