Behçet syndrome is a systemic vasculitis with an unknown aetiology affecting the small and large vessels of the venous and arterial systems. The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn’s disease suggest that multiple pathological pathways are involved in Behçet syndrome. These disease features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 as a genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bona fide disease, especially in non-endemic regions, suggests that other factors must also be operative in Behçet syndrome. Despite lacking a clear aetiological mechanism and definition, management of manifestations that include major vascular disease, eye disease and central nervous system involvement has improved with the help of new technology. Furthermore, even with our incomplete understanding of disease mechanisms, the prognoses of patients with Behçet syndrome, including those with eye disease, continue to improve. New treatment options and a better understanding of the underlying pathogenesis for various manifestations of this condition are required to further improve the management of the disease, which will improve patient quality of life.

Behçet 综合征是一种系统性血管炎，病因不明，影响静脉和动脉系统的大小血管。症状群的存在、疾病表达的区域差异以及与克罗恩病的相似性表明 Behçet 综合征涉及多种病理途径。这些疾病特征也使制定疾病标准变得困难。遗传研究已将HLA-B*51 确定为遗传风险因素。然而，HLA-B*51的低流行率在许多真正的疾病患者中，特别是在非流行地区，表明其他因素也必须在 Behçet 综合征中起作用。尽管缺乏明确的病因机制和定义，但在新技术的帮助下，对包括大血管疾病、眼部疾病和中枢神经系统受累在内的表现的管理有所改善。此外，即使我们对疾病机制的了解不完全，白塞综合征患者（包括眼病患者）的预后仍在不断改善。需要新的治疗方案和更好地了解这种疾病各种表现的潜在发病机制，以进一步改善疾病的管理，从而提高患者的生活质量。