Division of Rheumatology, Department of Internal Medicine, Istanbul University-Cerrahpasa, School of Medicine, and Behçet's Disease Research Centre, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.
Clin Exp Rheumatol. 2024 Oct;42(10):1999-2007. doi: 10.55563/clinexprheumatol/fqao16. Epub 2024 Oct 15.
The aim of this review is to provide a critical summary of studies published during 2023 that contribute to our understanding of Behçet's syndrome. An increase in the incidence of BS was reported in Northern Spain after 2014.Studies on patient perspectives showed the impact of Behçet's syndrome on quality of life, daily activities, education, work, and relationships. Differences in genetics among Behçet's syndrome patients with different types of organ involvement were reported and an association between HLA-B/MICA and SLCO4A1 polymorphisms and eye involvement and between DDX60L polymorphisms and nervous system involvement were observed. It was suggested that Integrin α9β1 plays a crucial role in the neutrophil-mediated inflammatory pathways underlying this syndrome and the dysfunction of the PDL1/PD-1 pathway may be associated with the hyperactivity of the Th-1/Th-17 axis. Vein wall thickness seems to be increased in patients with Behçet's syndrome, but its pathogenetic and clinical implications are not clear. There is growing evidence regarding the efficacy of TNF inhibitors in different types of organ involvement. A number of small case series point out to the potential role of TCZ and JAK inhibitors.
本综述的目的是批判性地总结 2023 年发表的有助于我们理解贝切特综合征的研究。报告称，2014 年后西班牙北部的 BS 发病率有所增加。对患者观点的研究表明，贝切特综合征对生活质量、日常活动、教育、工作和人际关系有影响。报告了不同类型器官受累的贝切特综合征患者之间的遗传差异，并观察到 HLA-B/MICA 和 SLCO4A1 多态性与眼部受累之间以及 DDX60L 多态性与神经系统受累之间的关联。有人提出整合素α9β1在该综合征中性粒细胞介导的炎症途径中起关键作用，PDL1/PD-1 途径的功能障碍可能与 Th-1/Th-17 轴的过度活跃有关。贝切特综合征患者的静脉壁厚度似乎增加，但它的发病机制和临床意义尚不清楚。越来越多的证据表明 TNF 抑制剂在不同类型的器官受累中有效。一些小的病例系列指出 TCZ 和 JAK 抑制剂的潜在作用。
