在NLRP3相关的自身炎症性疾病中发现了新的视网膜发现
Zhangwanyu Wei1,2, Zhikun Yang1,2, Donghui Li1,2
1Department of Ophthalmology, Peking Union Medical College Hospital, Beijing, 100730, China.
Orphanet journal of rare diseases
|July 21, 2023
概括
罕见的NLRP3-相关的自身炎症性疾病 (NLRP3-AID) 可以导致显著的眼睛问题,包括新的视网膜发现,如血管泄漏和缺血. 定期进行眼科检查对于这些患者的早期检测和治疗至关重要.
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:
- 与NLRP3相关的自身炎症性疾病 (NLRP3-AID) 是一种罕见的全身性炎症性疾病.
- 在NLRP3-AID的眼部表现没有很好地描述.
- 了解眼睛参与的频谱对于患者管理至关重要.
研究的目的:
- 调查NLRP3-AID与视网膜变化之间的关联.
- 评估NLRP3-AID患者眼部干扰的患病率和类型.
主要方法:
- 对20名NLRP3-AID患者进行了回顾性队列研究.
- 综合眼科检查,包括视力敏度,IOP,裂纹灯, fundus摄影,OCT和血管学.
- 对眼睛和视网膜发现的分析.
主要成果:
- 65%的患者 (13/20) 显示眼部受影响.
- 常见的表现包括结膜炎, papilledema,视网膜病变和视力缩.
- 25%的患者 (5/20) 的视网膜新发现包括血管泄漏,微动脉瘤,黄斑缺血症和干燥.
结论:
- NLRP3-AID与各种眼部表现有关.
- 周围视网膜血管泄漏,黄斑缺血,微动脉瘤和瘤是新发现的视网膜发现.
- 对于患有NLRP3-AID的患者,建议进行详细的视网膜检查.
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