医学
疾病
病因学
白塞病
机制(生物学)
病态的
人类白细胞抗原
重症监护医学
生物信息学
免疫学
病理
抗原
生物
认识论
哲学
作者
Hasan Yazıcı,Emire Seyahi,Gülen Hatemi,Yusuf Yazıcı
标识
DOI:10.1038/nrrheum.2017.208
摘要
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 Behcet 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 Behcet 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 Behcet syndrome, including those with eye disease, continue to improve.
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