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Concept: HLA-B51

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The clinical manifestations of Behcet disease (BD) have been reported to differ according to country, region, and race. Gender, onset age, and human leukocyte antigen (HLA)-B51 have also been known as the factors that influence the clinical features of BD. The aim of this study is to investigate the clinical phenotypes of Korean patients who visited the rheumatology clinic with BD with respect to gender, onset age, and HLA-B51.

Concepts: Immune system, Medicine, Death, Human leukocyte antigen, Hangul, Behçet's disease, Hulusi Behçet, HLA-B51

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Behcet’s disease (BD) is a multisystemic chronic inflammatory disorder that presents throughout the world with high frequency in Turkey and Middle East. BD has been shown to be associated with genotoxicity as patients with the disease have demonstrated high rates of sister chromatid exchange (SCE) and oxidative DNA damage. In this study, we examined the effect of vitamin E, which is known for its strong antioxidant activity, on the rate of SCE in cultured lymphocytes obtained from BD patients. In addition, the susceptibility of patient lymphocytes to the mutagenic agent mitomycin C (MMC) was also investigated. The results showed significant elevation in the rate of SCE in lymphocytes obtained from patients compared to those from healthy subjects (P < 0.01). Treatment with vitamin E normalized the elevated rate of SCE to a comparable level observed in the control group (P < 0.01). Finally, treatment of cultures with MMC significantly increased the rate of SCE in the lymphocytes of both patients and controls (P < 0.001). The magnitude of change in the rate of SCE induced by MMC was equivalent in both groups. This result suggests similar sensitivity of BD lymphocytes and control ones to MMC. In conclusion, genotoxicity associated with BD can be overcome by treatment with vitamin E. Lymphocytes of BD have normal sensitivity to the mutagenic agent MMC.

Concepts: Scientific method, Mutation, Antioxidant, Oxidative stress, Middle East, Vitamin C, Mutagen, HLA-B51

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Objective: To report the long-term outcome of neurological involvement in patients with Behçet’s disease (BD). Methods: Retrospective analysis of 115 patients fulfilling the international criteria for BD [57% male with median [Q1-Q3] age of 37 [30-46] years] and with neuro-Behçet’s disease (NBD) after exclusion of cerebral venous thrombosis. Factors associated with relapse of NBD, dependence and mortality were assessed. Results: Seventy-eight (68%) patients presented with acute NBD and 37 (32%) with a progressive course. The HLA B51 allele was carried by 49% of patients. Overall, 46/115 (40%) patients had a severe baseline disability status, represented by a Rankin score ≥ 3. The 5 and 7 years event-free survival rates were 65% and 53%, respectively. In multivariate analysis, a positive HLA-B51 status was independently associated with the risk of NBD relapse (OR=3.6 [1.5-9.1]). After a median follow-up of 73 [59-102] months, 21 (25.2%) patients became dependent or died. Factors independently associated with poor outcome were paresis at onset (OR=6.47 [1.73-24.23]) and a brainstem location of inflammatory lesions on MRI (OR=8.41 [1.03-68.43]). All 115 patients were treated with glucocorticosteroids, including 53/115 (46.1%) with cyclophosphamide and 40/115 (34.8%) with azathioprine. A trend towards longer event-free survival was observed in severe NBD patients (i.e. Rankin score ≥ 3 at onset) receiving intravenous cyclophosphamide compared with those treated with azathioprine (p =0.06). Conclusion: NBD is a severe condition in which patients with the HLA-B51 allele appear to experience a worse prognosis. © 2013 American College of Rheumatology.

Concepts: Death, Rheumatoid arthritis, Modified Rankin Scale, Multivariate statistics, Disability, Deep vein thrombosis, Dependency, HLA-B51