背景:Susac综合征是一种血管病变,导致经典的三联征视网膜分支动脉阻塞(BRAO),内耳缺血,和脑缺血。在这篇回顾性图表回顾中,我们描述了荧光素血管造影(FA)的结果和其他辅助研究在Susac综合征,包括持续性疾病活动的出现和FA上新的亚临床疾病的发生。
方法:这个多中心,回顾性病例系列是机构审查委员会批准的,包括使用FA评估的Susac综合征完全三联症患者,对比大脑的MRI,和2010年至2020年的听力测量。对这些辅助检查的医疗记录进行了审查,随着人口统计学,症状,视敏度,视野缺陷,和眼底镜检查的结果。临床复发被定义为在初始诱导临床静止后的随访期间疾病活动的任何客观证据。主要结果测量是辅助测试的灵敏度,包括FA,MRI,和测听法,检测复发。
结果:31例患者中有20例(64%)具有完整的脑三联征,视网膜,Susac综合征和前庭耳蜗受累。诊断时的中位年龄为43.5岁(范围21-63),14名(70%)是女性。听力损失发生在20(100%),13例脑病(65%),15岁的眩晕(75%),在整个随访过程中,有19例(95%)头痛。两只眼睛在开始和最后一次访问时的中位视力为20/20。17(85%)在基线时患有BRAO,10人(50%)在随访期间经历了随后的BRAO。FA在20例(100%)中显示了来自先前小动脉损伤的非特异性渗漏,包括其他情况缓解的患者。在进行所有测试方式的11次疾病活动中,视野测试/眼底镜检查异常4例(36.4%),头颅MRI2例(18.2%),8人中的听力图(72.7%),和FA在9(81.8%)。
结论:新的FA渗漏是活动性疾病最敏感的标志物。持续泄漏表示以前的损坏,而新的渗漏区域提示持续的疾病活动,需要考虑修改免疫抑制治疗.
BACKGROUND: Susac syndrome is a vasculopathy, resulting in the classic triad of branch retinal artery occlusion (BRAO), inner ear ischemia, and brain ischemia. In this retrospective chart review, we characterize fluorescein angiography (FA) findings and other ancillary studies in Susac syndrome, including the appearance of persistent disease activity and the occurrence of new subclinical disease on FA.
METHODS: This multicenter, retrospective
case series was institutional review board-approved and included patients with the complete triad of Susac syndrome evaluated with FA, contrasted MRI of the brain, and audiometry from 2010 to 2020. The medical records were reviewed for these ancillary tests, along with demographics, symptoms, visual acuity, visual field defects, and findings on fundoscopy. Clinical relapse was defined as any objective evidence of disease activity during the follow-up period after initial induction of clinical quiescence. The main outcome measure was the sensitivity of ancillary testing, including FA, MRI, and audiometry, to detect relapse.
RESULTS: Twenty of the 31 (64%) patients had the complete triad of brain, retinal, and vestibulocochlear involvement from Susac syndrome and were included. Median age at diagnosis was 43.5 years (range 21-63), and 14 (70%) were women. Hearing loss occurred in 20 (100%), encephalopathy in 13 (65%), vertigo in 15 (75%), and headaches in 19 (95%) throughout the course of follow-up. Median visual acuity at both onset and final visit was 20/20 in both eyes. Seventeen (85%) had BRAO at baseline, and 10 (50%) experienced subsequent BRAO during follow-up. FA revealed nonspecific leakage from previous arteriolar damage in 20 (100%), including in patients who were otherwise in remission. Of the 11 episodes of disease activity in which all testing modalities were performed, visual field testing/fundoscopy was abnormal in 4 (36.4%), MRI brain in 2 (18.2%), audiogram in 8 (72.7%), and FA in 9 (81.8%).
CONCLUSIONS: New leakage on FA is the most sensitive marker of active disease. Persistent leakage represents previous damage, whereas new areas of leakage suggest ongoing disease activity that requires consideration of modifying immunosuppressive therapy.