Tagami Mizuki, Honda Shigeru, Azumi Atsushi
Department of Ophthalmology and Visual Sciences, Graduate School of Medicine, Osaka City University, Osaka, Japan.
Ophthalmology Department and Eye Center, Kobe Kaisei Hospital, Kobe, Hyogo, Japan.
Clin Ophthalmol. 2022 Mar 18;16:841-850. doi: 10.2147/OPTH.S284609. eCollection 2022.
Dysthyroid optic neuropathy (DON) is a potentially sight-threatening eye disease associated with Graves' orbitopathy (GO). DON is not common in GO patients, reportedly occurring in only about 5% of patients. The pathogenesis of severe DON is considered to involve both muscular nerve strangulation and impaired blood flow. There is some objective grading of physical examination findings and the severity of GO, including a clinical activity score (CAS) and EUropean Group On Graves' Orbitopathy (EUGOGO), but no specialized protocol completely characterizes DON. Most clinicians have decided that the combination of clinical activity findings, including visual acuity, color vision, and central critical fusion frequency, and radiological findings, including magnetic resonance imaging (MRI), can be used to diagnose DON. MRI has the most useful findings, with T2-weighted and fat-suppressed images using short-tau inversion recovery (STIR) sequences enabling detection of extraocular changes including muscle and/orbital fat tissue swelling and inflammation and, therefore, disease activity. The first-choice treatment for DON is intravenous administration of steroids, with or without radiotherapy. Unfortunately, refractoriness to this medical treatment may indicate the need for immediate orbital decompression within 2 weeks. Especially in the acute phase of DON, thyroid function is often unstable, and the surgeon must always assume the risk of general anesthesia and intra- and post-operative management. In addition, there are currently many possible therapeutic options, including molecular-targeted drugs. The early introduction and combination of these immunomodulators, including Janus kinase inhibitors and insulin-like growth factor-1 receptor antibody (teprotumumab), may be effective for GO with DON. However, this is still under investigation, and the number of case reports is small. It is possible that these options could reduce systemic adverse events due to unfocused glucocorticoid administration. The pathophysiology of DON is not yet fully understood, and further studies of its treatment and long-term visual function prognosis are needed.
甲状腺功能异常性视神经病变（DON）是一种与Graves眼病（GO）相关的、可能威胁视力的眼部疾病。DON在GO患者中并不常见，据报道仅约5%的患者会发生。严重DON的发病机制被认为涉及肌肉神经绞窄和血流受损。对于体格检查结果和GO的严重程度有一些客观分级，包括临床活动评分（CAS）和欧洲Graves眼病研究组（EUGOGO），但没有专门的方案能完全表征DON。大多数临床医生认为，结合包括视力、色觉和中心临界融合频率在内的临床活动结果以及包括磁共振成像（MRI）在内的放射学结果，可用于诊断DON。MRI的结果最有用，使用短反转时间反转恢复（STIR）序列的T2加权和脂肪抑制图像能够检测眼外变化，包括肌肉和/或眶脂肪组织肿胀和炎症，从而判断疾病活动情况。DON的首选治疗方法是静脉注射类固醇，可联合或不联合放疗。不幸的是，对这种药物治疗的难治性可能表明需要在2周内立即进行眼眶减压。特别是在DON的急性期，甲状腺功能往往不稳定，外科医生必须始终承担全身麻醉以及术中及术后管理的风险。此外，目前有许多可能的治疗选择，包括分子靶向药物。这些免疫调节剂，包括Janus激酶抑制剂和胰岛素样生长因子-1受体抗体（teprotumab）的早期引入和联合使用，可能对伴有DON的GO有效。然而，这仍在研究中，病例报告数量较少。这些选择有可能减少因无针对性使用糖皮质激素导致的全身不良事件。DON的病理生理学尚未完全了解，需要对其治疗和长期视觉功能预后进行进一步研究。
