Susac syndrome is a rare microangiopathy of suspected autoimmune origin affecting arteries of the retina, the cochlea and the brain. The aim of the study was to give a review of the disease entity and determine the proportion of cases and their characteristics in a uveitis referral centre. Charts of patients with the diagnosis of Susac syndrome seen in the Uveitis Clinic of the Centre for Ophthalmic Specialised Care (COS), Lausanne, Switzerland were reviewed retrospectively to determine the frequency of such cases in a uveitis referral centre. Clinical symptoms and signs, functional data, imaging signs and evolution were analysed in the 3 COS cases and one case shared with the Uveitis Clinic of the Department of Ophthalmology, University of Innsbruck, Austria. Characteristic signs were searched possibly allowing a prompt diagnosis. During the period from 1994 to 2019 (24 years, 2045 patients), 3 charts with the diagnosis of Susac syndrome were found (0.15%). The whole collective, including the additional case, comprised three women aged 28, 32 and 63 at presentation and one man, aged 42. None of the 3 cases that were referred were diagnosed beforehand. The characteristic item found in all 4 cases was the abrupt arterial stop or segmental interruption of arteries and increased staining of arterial wall on angiography more clearly shown on indocyanine green angiography that can potentially be proposed as a crucial diagnostic element. All 4 cases responded to dual steroidal and non-steroidal immunosuppression. Under treatment, all four patients did not show any further evolution. Susac syndrome is a multilocation arteritis of the head that can involve the eye, ear and brain often first diagnosed by the ophthalmologist. The diagnosis is rapidly reached in uveitis referral centres but seems to be missed otherwise, A helpful angiographic sign to be searched is an abrupt or segmental arterial stop and increased staining of the arterial wall more clearly seen on indocyanine green angiography. Patients often present first to the ophthalmologist who should be acting as a whistleblower to avoid severe involvement of the brain.

中文翻译：

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Susac综合征（视网膜-耳蜗-脑血管炎），眼科医生在举报者的作用

Susac综合征是一种罕见的疑似自身免疫起源的微血管病，可影响视网膜，耳蜗和大脑的动脉。该研究的目的是对葡萄膜炎转诊中心的疾病实体进行审查，并确定病例的比例及其特征。回顾性地回顾了瑞士洛桑眼科专业护理中心（COS）葡萄膜炎诊所中诊断为Susac综合征的患者图表，以确定在葡萄膜炎转诊中心发生此类病例的频率。分析了3例COS病例的临床症状和体征，功能数据，影像学征象和演变，其中1例与奥地利因斯布鲁克大学眼科的葡萄膜炎诊所共享。搜索了特征性标志，可能有助于迅速诊断。在1994年至2019年期间（24年，2045例患者），发现了3幅诊断为Susac综合征的图表（0.15％）。包括另外一例在内的整个集体包括三名分别为28岁，32岁和63岁的女性和一名42岁的男性。被转诊的3例病例均未事先诊断。在所有4例病例中发现的特征性项目是在血管造影术上突然停止动脉或节段性中断以及动脉壁染色增加，在吲哚菁绿血管造影术上更清楚地显示了这一点，这可能被认为是至关重要的诊断要素。所有4例均对类固醇和非类固醇双重免疫抑制反应。在治疗下，所有四名患者均未显示出进一步的进展。Susac综合征是头部的多部位动脉炎，可能累及眼睛，耳朵和大脑通常最先由眼科医生诊断。在葡萄膜炎转诊中心可以很快达到诊断，但似乎没有被漏诊。要搜索的有用的血管造影迹象是在吲哚菁绿血管造影上更清楚地看到动脉突然或节段性停止以及动脉壁染色增加。患者通常首先是眼科医生，应充当举报人，以避免严重的大脑受累。