The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest multiple pathological pathways are involved in Behçet syndrome. These features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 to be the important genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bone fide disease, especially in non-endemic regions, suggests other factors must also be operative in Behçet syndrome. This consideration is also true for the newly proposed 'MHC-I-opathy' concept. Despite lacking a clear aetiological mechanism and definition, management of manifestations that include major vascular disease (such as Budd–Chiari syndrome and pulmonary artery involvement), 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.

症状群的存在，疾病表达的区域差异以及与例如克罗恩病的相似性提示Behçet综合征涉及多种病理途径。这些特征也使制定疾病标准变得困难。遗传研究确定HLA-B * 51是重要的遗传危险因素。然而，许多骨质疏松患者的HLA-B * 51患病率较低这种疾病，特别是在非流行地区，提示在Behçet综合征中还必须治疗其他因素。对于新提出的“ MHC-I-病”概念也是如此。尽管缺乏明确的病因学机制和定义，但在新技术的帮助下，包括主要血管疾病（如Budd–Chiari综合征和肺动脉受累），眼部疾病和中枢神经系统受累等表现的管理得​​到改善。此外，即使我们对疾病的机理还不完全了解，但Behçet综合征患者（包括眼疾患者）的预后仍在不断改善。