ABSTRACT

Purpose

Susac’s syndrome (ScS) is a rare, potentially life-threatening auto-immune disease. Ophthalmic imaging can depict characteristic branch retinal arteriolar occlusions (BRAO) and arterial wall hyperfluorescences that form one of the three diagnostic pillars of this condition. We aim to demonstrate that ophthalmological ultrawide-field (UWF) imaging allows for a qualitative and quantitative assessment in ScS, with application in diagnostics, monitoring of treatment response, and titration of therapy.

Methods

In seven ScS patients (♀:♂ = 2:5), with a median age of 36 years, range 18 to 57 years, serial ultrawide-field fluoresceine angiography (UWF-FA) studies were performed, with adjunctive wide-field optical coherence tomography angiography analyses (WF-OCTA) in five patients. Mean follow-up was 12.5 months, range 1 to 46 months.

Results

In all seven patients, BRAO and arterial wall hyperfluorescences were present in UWF-FA and calculated as a quantitative score of disease activity in fluorescein angiography (DA-FA) during follow-up visits. Treatment response was accessible in follow-up imaging as partial reperfusion of retinal vessels, resolution of arterial wall hyperfluorescences and consequently, in reduction of DA-FA score. While qualitative analysis of WF-OCTA provided further information about retinal micro-perfusion, quantitative analysis did not demonstrate a vectored treatment response as it was accessible in FA.

Conclusion

DA-FA score, as a comprehensive disease activity parameter in ScS has potential to facilitate optimal communication between subspecialties and thereby treatment success.

中文翻译：

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基于超广角成像的Susac综合征疾病活动定量评估新方法

摘要

目的

Susac 综合征 (ScS) 是一种罕见的、可能危及生命的自身免疫性疾病。眼科成像可以描绘特征性的视网膜分支小动脉闭塞 (BRAO) 和动脉壁高荧光，它们构成了这种情况的三个诊断支柱之一。我们的目标是证明眼科超广角 (UWF) 成像允许对 ScS 进行定性和定量评估，并应用于诊断、治疗反应监测和治疗滴定。

方法

在 7 名 ScS 患者 (♀:♂ = 2:5) 中，中位年龄为 36 岁，范围为 18 至 57 岁，进行了系列超广角荧光血管造影 (UWF-FA) 研究，并辅以广角光学相干性5 名患者的断层扫描血管造影分析 (WF-OCTA)。平均随访时间为 12.5 个月，范围为 1 至 46 个月。

结果

在所有 7 名患者中，BRAO 和动脉壁高荧光均存在于 UWF-FA 中，并在随访期间计算为荧光素血管造影 (DA-FA) 中疾病活动的定量评分。在后续成像中可以得到治疗反应，因为视网膜血管的部分再灌注、动脉壁高荧光的消退以及因此 DA-FA 评分的降低。虽然 WF-OCTA 的定性分析提供了有关视网膜微灌注的更多信息，但定量分析并未证明矢量治疗反应，因为它在 FA 中是可访问的。

结论

DA-FA 评分作为 ScS 中的综合疾病活动参数，有可能促进亚专业之间的最佳沟通，从而促进治疗成功。