Division of Ophthalmology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Department of Ear, Nose and Throat, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Eye (Lond). 2022 Apr;36(4):781-788. doi: 10.1038/s41433-021-01464-7. Epub 2021 Apr 20.
Susac's syndrome, a rare autoimmune vasculo-occlusive disease, may pose a diagnostic challenge and result in a devastating ocular and systemic outcome. Our study identifies a new retinal finding and evaluates disease outcome. We aimed to assess clinical and imaging findings, systemic manifestations and disease outcome in patients with ocular Susac's syndrome under immunosuppressive/immunomodulation therapies.
Retrospective tertiary center study including patients with a diagnosis of Susac's syndrome with >12 months follow up. Medical record review including ocular, neurological and auditory clinical and imaging findings, and treatment modalities. Main outcome measures were clinical manifestations and disease outcome.
Seven patients (14 eyes) with a mean age of 34.1 years were included. Mean follow-up was 31.9 months (12.4-72.4). All had bilateral ocular disease. Retinal microaneurysms, a new ocular finding, were demonstrated in 5 patients and persisted at the final visit. In 5 eyes, they further extended during follow-up. All were treated with immunosuppressive drugs and 5/7 additional immunomodulation therapy. At last examination, best corrected visual acuity was >20/40 in all eyes, 1/10 eyes had visual field deterioration, no eye had active ocular disease, all patients achieved neurological stability, and 1 patient had auditory deterioration.
Retinal microaneurysms, a new ocular finding in Susac's syndrome, were present in most of our patients, indicating ischemic retinal damage. Immunosuppressive and immunomodulation therapies seem to be highly effective in the control of disease activity.
Susac 综合征是一种罕见的自身免疫性血管闭塞性疾病，可能具有诊断挑战性，并导致严重的眼部和全身后果。我们的研究确定了一种新的视网膜表现，并评估了疾病结局。我们旨在评估接受免疫抑制/免疫调节治疗的眼部 Susac 综合征患者的临床和影像学表现、全身表现和疾病结局。
这是一项回顾性的三级中心研究，纳入了诊断为 Susac 综合征且随访时间超过 12 个月的患者。对病历进行了回顾，包括眼部、神经和听觉的临床和影像学表现以及治疗方式。主要观察指标是临床表现和疾病结局。
纳入了 7 例（14 只眼）年龄平均为 34.1 岁的患者。平均随访时间为 31.9 个月（12.4-72.4）。所有患者均有双眼疾病。5 例患者出现了视网膜微动脉瘤，这是一种新的眼部表现，在最后一次就诊时仍存在。在 5 只眼中，它们在随访过程中进一步扩展。所有患者均接受了免疫抑制药物治疗，5/7 例患者还接受了免疫调节治疗。在最后一次检查时，所有患者的最佳矫正视力均>20/40，1/10 只眼的视野恶化，无眼有活动性眼部疾病，所有患者的神经功能稳定，1 例患者的听力恶化。
视网膜微动脉瘤是 Susac 综合征的一种新的眼部表现，在我们的大多数患者中存在，表明存在缺血性视网膜损伤。免疫抑制和免疫调节治疗似乎对控制疾病活动非常有效。
