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主要系统性血管炎相关视神经炎:单中心 10 年回顾性分析。

The primary systemic vasculitis associated optic neuritis: a retrospective analysis in a single center over 10 years.

机构信息

Department of Ophthalmology, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, China.

Department of Rheumatology, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, China.

出版信息

Int Ophthalmol. 2024 Sep 23;44(1):387. doi: 10.1007/s10792-024-03307-2.

Abstract

OBJECTIVES

To investigate the clinical and image characteristics of primary systemic vasculitis-associated optic neuritis patients.

METHODS

This is a retrospective study. The patients clinically diagnosed with primary system vasculitis-induced optic neuritis were recruited from March 2013 to December 2023. All cases received orbital magnetic resonance imaging scans were analyzed. The ocular findings, systemic manifestations, laboratory data and prognosis were reviewed retrospectively. In addition, the related literature was reviewed.

RESULTS

Fourteen patients (21 eyes), including 10 men and 4 women, were enrolled in this study. The ages ranged from 30 to 86 years in this cohort. Orbits MRI detects the enlargement and/or enhancement of the optic nerve. Cases 1-5 reported a confirmed diagnosis of Takayasu's arteritis, and cases 6-8 had giant cell arteritis. Cases 9-13 were antineutrophil cytoplasmic antibody-associated vasculitis. Case 14 was Cogan's syndrome. Mult organs and tissues, such as the kidneys, heart, paranasal sinuses, meninges, and respiratory system, were involved. In all of the 14 involved patients, the disease onset was either during the fall or winter season. There were no or only slight improvements in visual activity after conventional therapies.

CONCLUSIONS

The autoantibodies' attack on the optic nerve, ischemic damage, or destruction of the blood-brain barrier may be the potential pathogenesis of vasculitis-associated optic neuritis. Even with prompt and aggressive clinical interventions, the prognosis remains unsatisfactory.

摘要

目的

研究原发性系统性血管炎相关性视神经炎患者的临床和影像特征。

方法

这是一项回顾性研究。从 2013 年 3 月至 2023 年 12 月,招募了经临床诊断为原发性系统性血管炎引起的视神经炎的患者。对所有接受眼眶磁共振成像扫描的病例进行了分析。回顾性分析了眼部表现、全身表现、实验室数据和预后。此外,还回顾了相关文献。

结果

本研究纳入了 14 例(21 只眼)患者,包括 10 例男性和 4 例女性。该队列的年龄为 30-86 岁。眼眶 MRI 检测到视神经增大和/或增强。病例 1-5 报告了明确的 Takayasu 动脉炎诊断,病例 6-8 有巨细胞动脉炎。病例 9-13 为抗中性粒细胞胞质抗体相关性血管炎。病例 14 为 Cogan 综合征。肾脏、心脏、副鼻窦、脑膜和呼吸系统等多器官和组织受累。在所有 14 例受累患者中,疾病发作均在秋季或冬季。常规治疗后,视力活动几乎没有改善或只有轻微改善。

结论

自身抗体对视神经的攻击、缺血损伤或血脑屏障的破坏可能是血管炎相关性视神经炎的潜在发病机制。即使进行了及时和积极的临床干预,预后仍不理想。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d30/11420302/0c9ab14f88d4/10792_2024_3307_Fig1_HTML.jpg

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