AB0556 HLA Revisited in Egyptian Patients with Behçet's Syndrome:New Associations of Hla Alleles with Susceptibility, Protection, Presentation and Severity of The Disaese

BackgroundBehçet's syndrome is a multisystem autoimmune syndrome that has a male predominance. Its manifestations usually start in the young adulthood with target organ damage affecting mainly the skin, eyes, Brain and blood vessels (1)ObjectivesThe aim of the study was to investigate HLA class I genotyping in a cohort of Egyptian patients with Behçet's syndrome and comparing them with HLA genotyping in healthy population (control group) to estimate possible association between HLA and syndrome susceptibility as well as syndrome presentations.MethodsFifty-seven Egyptian patients with Behçet's syndrome fulfilling the International study group criteria for Behçet's syndrome(2) were recruited from the Rheumatology department, Cairo University teaching hospitals.HLA class I genotyping was done for all patients via sequence specific oligonucleotides probes at the National Cancer Institute. HLA class I genotyping data of normal control group was obtained from case control studies done on Egyptian population with a total of 221 individual. (3–4)ResultsThe studied 57 patients were divided into 50 males and 7 females. The mean age of patients was 35.28±9.73 years with mean disease duration of 9.26±7.36 years. The main clinical features were oral ulcers (100%), genital ulcers (100%), eye involvement (54%) neurological involvement (29%) and vascular involvement (36%) furthermore (33%) had bilateral visual acuity ≤6/60 fulfilling the diagnosis of legal blindness.Certain HLA genotypes were significantly associated with susceptibility for Behçet's syndrome, the odds ratio (OR) for HLA-A68 was 8.4 (CI=2.9–25.9), HLA-B15 was 6.7 (CI=2.9–15.6), HLA-B51 was 6.6 (CI=3.4–13.0), HLA-A24 was 4.0 (CI=1.5–10.2) and HLA-A2 was 2.1 (CI=1.1–3.8). On the other hand HLA A3 genotype was found to be significantly protective with odds ratio of 0.003 (CI=0.01–0.6).HLA B51 was significantly associated with ocular disease with odds ratio of 3.47. Furthermore HLA B51 was associated with legal blindness with a significant odds ratio of 5.21.ConclusionsHLA A68, B15, B51, A24 and A2 are associated with Behçet's syndrome susceptibility in the present cohort of Egyptian patients and A3 was found to be protective.HLA B51 is associated with ocular involvement and more important the blinding eye disease and can be considered as a poor prognostic marker for ocular disease.ReferencesYazici, H., Seyahi, E., & Yurdakul, S. (2008). Behcet's syndrome is not so rare: why do we need to know?. Arthritis & Rh