Factor V Leiden mutation is associated with ocular involvement in Behçet disease.

PURPOSE: Behçet disease is a systemic disease of young adults characterized by venous occlusion in both the deep venous and retinal circulations. In severe ocular disease, blindness may occur despite immunosuppressive treatment. The most common inherited risk factor for the development of idiopathic venous thrombosis is the presence of the Factor V (FV Leiden) mutation, which confers resistance to activated protein C. The association of FV Leiden with Behçet disease has been reported, but its influence on ocular disease is not known. We therefore investigated the prevalence of this mutation in patients with Behçet disease to determine its contribution to the presence and severity of ocular disease. METHODS: One hundred and six Middle Eastern patients satisfying international criteria, and 120 healthy control subjects without a history of venous thrombosis were included in the study, and patients underwent standard examination by two ophthalmologists with an interest in Behçet disease. Genomic DNA was extracted from peripheral blood leukocytes and screened for the FV Leiden mutation with the polymerase chain reaction method with sequence-specific primers (PCR-SSP). RESULTS: FV Leiden was detected in 19% (23/120) of the control population compared with 27% (29/106) of all patients with Behçet disease (P = .13). However, among patients with Behçet disease who had ocular disease (75/106), the prevalence of FV Leiden was significantly higher (32%) than it was in control subjects (P = .04). Furthermore, ocular patients with Behçet disease in whom retinal occlusive disease was observed (25/75) had the highest expression of FV Leiden (44%). CONCLUSIONS: These data suggest that FV Leiden may be an additional risk factor for the development of ocular disease and, in particular, retinal vaso-occlusion, and it may contribute to the poor visual outcome in these patients.

Soluble adhesion molecules in Behçet's disease.

BACKGROUND: Behçet's disease (BD) may lead to blindness in up to 25% of eyes. Soluble (s)ICAM-1 but not sVCAM-1 is associated with relapse in idiopathic uveoretinitis and is reported to be raised in BD patients. We have investigated the levels of sICAM-1 and sVCAM-1 in Palestinian patients with BD and related them to both ocular and systemic disease activity and to immunosuppressive treatment. METHODS: A total of 51 patients (43 male, 8 female; mean ages 29.8 & 31.9 yr) were examined at the St John Ophthalmic Hospital during a one year period (135 consultations). Disease activity was determined from history and standard ocular examination. Anterior uveitis, vitritis and retinal vasculitis acted as markers of ocular inflammation. Peripheral venous sICAM-1 and sVCAM-1 levels were determined by standard ELISA. A total of 53 healthy age- and sex-matched clinic staff members acted as controls. RESULTS: sICAM-1 and sVCAM-1 were both significantly lower in patients on systemic immunosuppression than in those off treatment (p < 0.001). Among patients off systemic treatment, sICAM was higher in the group with active systemic disease but quiet eyes (p = 0.003), but not in those with active ocular disease (p = 0.09), compared to controls. sVCAM was not raised in either group. CONCLUSION: Systemic immunosuppression was associated with reduced sICAM-1 and sVCAM-1, supporting a role for adhesion molecules in the pathogenesis of BD. sICAM-1 levels were raised in association with inflammatory features implicating endothelial activation in active BD. The mean sICAM-1 was higher in active ocular patients than controls.

Lens aspiration.

Since the King Hussein Medical Centre was opened in 973, lens aspiration has been adopted as the standard method of treating cataracts in children and young people under 30 years of age, and has been used in a personal series of 126 eyes of 88 patients. The results support the opinion that the surgical technique of continuous infusion with a two-way cannula is a satisfactory method of treating soft cataract.

Different B lymphocyte alloantigens associated with multiple sclerosis in Arabs and North Europeans.

The frequencies of HLA and B-lymphocyte alloantigens were investigated in 32 Arab patients with clinically definite or early probable multiple sclerosis (M.S.) and compared with those found in 43 healthy Arab controls. A significant association was found between M.S. and the B-lymphocyte alloantigen, BT 102; this contrasts with previous findings of an association between the B-lymphocyte alloantigen BT 101 and M.S. in North Europeans. It is suggested that this difference is due to the involvement of different environmental agents, possibly viruses, in the pathogenesis of M.S. in these populations.