Papasavvas Ioannis, Teuchner Barbara, Herbort Carl Peter
Retinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), Clinic Montchoisi Teaching Centre, Lausanne, Switzerland.
Department of Ophthalmology, University of Innsbruck, Innsbruck, Austria.
J Ophthalmic Inflamm Infect. 2020 Oct 30;10(1):27. doi: 10.1186/s12348-020-00217-z.
BACKGROUND/PURPOSE: 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.
背景/目的：Susac综合征是一种罕见的微血管病，怀疑起源于自身免疫，可累及视网膜、耳蜗和脑动脉。本研究的目的是对该疾病实体进行综述，并确定葡萄膜炎转诊中心的病例比例及其特征。
回顾性分析瑞士洛桑眼科专科护理中心（COS）葡萄膜炎诊所诊断为Susac综合征的患者病历，以确定葡萄膜炎转诊中心此类病例的发生率。对3例COS病例以及与奥地利因斯布鲁克大学眼科系葡萄膜炎诊所共享的1例病例的临床症状和体征、功能数据、影像学表现及病情进展进行了分析。寻找可能有助于快速诊断的特征性表现。
在1994年至2019年期间（24年，共2045例患者），发现3例诊断为Susac综合征的病历（0.15%）。包括额外病例在内的全部病例中，有3名女性，就诊时年龄分别为28岁、32岁和63岁，1名男性，年龄为42岁。转诊的3例病例均未预先诊断。在所有4例病例中发现的特征性表现是动脉造影时动脉突然中断或节段性中断，以及动脉壁染色增强，吲哚菁绿血管造影更清晰地显示了这一点，这可能被视为关键的诊断要素。所有4例病例对甾体和非甾体联合免疫抑制治疗均有反应。治疗期间，所有4例患者均未出现进一步进展。
Susac综合征是一种头部多部位动脉炎，可累及眼、耳和脑，通常由眼科医生首先诊断。在葡萄膜炎转诊中心可迅速做出诊断，但在其他情况下似乎易被漏诊。一个有用的血管造影表现是动脉突然或节段性中断，以及动脉壁染色增强，吲哚菁绿血管造影更清晰可见。患者通常首先就诊于眼科医生，眼科医生应发挥预警作用，以避免脑部严重受累。
