Behçet's disease in Tunisia. Demographic, clinical and genetic aspects in 260 patients.

To analyze demographic, clinical and genetic features of Behçet's disease (BD) in Tunisia and to compare them with other ethnic and geographic groups.Two hundred and sixty patients with BD (International Study Group criteria) received in the Department of Internal Medicine of the University Hospital La Rabta in Tunisia, from 1987 to 2006, were retrospectively studied. Demographic, clinical, and genetic data were recorded and analyzed using SPSS 11.0.The cohort consisted of 188 males and 72 females (M/F = 2.61). The mean age at the onset of the disease was 29 years. Oral and genital ulcers (GU) were seen in 100% and 83% respectively. The most other common clinical features of BD were ocular involvement (51%), arthritis (38.8%), venous thrombosis (33%) and neuropsychiatric symptoms (24.2%). Only 1.5% had gastrointestinal lesions. HLA-B51 frequency was significantly higher in patients with BD (54% vs. 25.5% in healthy controls, p < 0.05). DVT and pseudofolliculitis were significantly more frequent in men whereas arthritis and erythema nodosum were significantly more frequent in women. DVT was also significantly more frequent in patients with GU and those with neurological involvement. GU, positive pathergy test and DVT were significantly less frequent in patients with ocular involvement. Neurological involvement consisted of 47 cases with CNS parenchymal lesions and 22 with cerebral vascular lesions (13 had both lesions); HLA B51 was significantly less frequent in patients with neurological involvement.Our series was characterized by particular aspects such as high frequency of DVT and neuropsychiatric involvement, and rare occurrence of gastrointestinal lesions. The results confirm the ethnic and geographic variation of BD expression.