常见的可变免疫缺陷和自身免疫性疾病:10年的单一中心经验
Filiz Sadi Aykan1, Fatih Çölkesen2, Recep Evcen3
1Department of Internal Medicine, Division of Clinical Immunology and Allergy, Ankara Health Sciences University, Gülhane Training and Research Hospital, Ankara, Türkiye.
Archives of rheumatology
|March 10, 2025
概括
患有常见变性免疫缺陷 (CVID) 和自身免疫性疾病 (ADs) 的成年人经历了更长的诊断延迟. 在CVID患者中,自身免疫性疾病更为频繁,因此需要提高医生意识.
科学领域:
- 免疫学 免疫学 免疫学
- 类风湿病学 类风湿病学
- 血液学 血液学 血液学
背景情况:
- 常见变性免疫缺陷 (CVID) 是一种主要免疫缺陷,其特征是免疫球蛋白水平低,B细胞功能受损.
- 在CVID患者中经常观察到自身免疫性疾病 (AD),影响其临床表现和预后.
- 了解CVID和AD之间的相互作用对于及时诊断和管理至关重要.
研究的目的:
- 为了确定CVID患者中ADS的频率.
- 评估CVID患者与没有ADS之间的临床,免疫学和器官表现差异.
- 评估ADS对CVID恶性瘤和死亡率的影响.
主要方法:
- 对85名CVID患者 (2013-2023) 的回顾性研究.
- 患者被分为AD-CVID (+) 和AD-CVID (-) 组.
- 临床特征,免疫学标志物,器官参与,恶性瘤和死亡率的比较.
主要成果:
- AD-CVID (+) 组的诊断延迟时间显著长 (84个月).
- 常见的AD包括细胞衰竭 (例如,免疫性血栓性紫外线) 和Sjögren综合征.
- 在AD-CVID (+) 组中观察到较低的类切换记忆B细胞水平;恶性瘤 (非霍奇金淋巴瘤) 更频繁.
结论:
- 患有AD的成人CVID患者面临诊断延迟.
- 自免疫性疾病在CVID中比在一般人群中更为普遍.
- 提高医生对CVID自身免疫表现的认识,对于减少诊断延迟至关重要.
相关概念视频
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The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
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