在VEXAS综合征中轨道/眼部炎症的参与:来自国际AIDA网络VEXAS注册表的数据
Antonio Vitale1, Valeria Caggiano1, Eduardo Martin-Nares2
1Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy; Rheumatology Unit, Policlinico "Le Scotte", Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, viale Bracci 16, Siena 53100, Italy.
Seminars in arthritis and rheumatism
|March 30, 2024
概括
轨道和眼部炎症在VEXAS综合征中很常见,特别是当与复发性多重合体炎相关时. 患有维克萨斯和眼睛炎症的患者需要更密切的监测,因为死亡风险增加.
科学领域:
- 眼科医生 眼科 眼科
- 类风湿病学 类风湿病学
- 遗传学 是一个遗传学.
背景情况:
- 维克萨斯综合征是最近发现的一种单源性自身炎症性疾病.
- 在VEXAS患者中经常观察到轨道和眼部炎症状况.
研究的目的:
- 在VEXAS综合征中详细说明轨道/眼部状况.
- 调查眼部表现和其他VEXAS疾病特征之间的关联.
主要方法:
- 对59名VEXAS患者的回顾性分析.
- 详细审查临床病史和眼睛/轨道发现.
- 统计分析以确定重要的关联.
主要成果:
- 45.8%的VEXAS患者表现出轨道/眼部炎症.
- 常见的表现包括眼周胀,表皮硬化,硬化,脑膜炎,结膜炎,白炎和轨道肌肉炎.
- 在VEXAS,轨道/眼部干扰和复发性多重合体炎 (RR 2.37,p=0.048) 之间发现了显著的关联.
- 6例死亡中有5例 (83.3%) 发生在有轨道/眼部炎症参与的患者中.
结论:
- 轨道/眼部炎症参与是VEXAS综合征的频繁和显著的表现.
- 复发性多重合体炎的存在增加了VEXAS眼部参与的风险.
- 眼科医生在诊断VEXAS综合征方面发挥着至关重要的作用.
- 由于死亡率较高,患有轨道/眼部炎症的VEXAS患者需要提高警和仔细跟踪.
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