Graves' orbitopathy (GO) is the main extrathyroidal manifestation of Graves' disease (GD). Choice of treatment should be based on the assessment of clinical activity and severity of GO. Early referral to specialized centers is fundamental for most patients with GO. Risk factors include smoking, thyroid dysfunction, high serum level of thyrotropin receptor antibodies, radioactive iodine (RAI) treatment, and hypercholesterolemia. In mild and active GO, control of risk factors, local treatments, and selenium (selenium-deficient areas) are usually sufficient; if RAI treatment is selected to manage GD, low-dose oral prednisone prophylaxis is needed, especially if risk factors coexist. For both active moderate-to-severe and sight-threatening GO, antithyroid drugs are preferred when managing Graves' hyperthyroidism. In moderate-to-severe and active GO i.v. glucocorticoids are more effective and better tolerated than oral glucocorticoids. Based on current evidence and efficacy/safety profile, costs and reimbursement, drug availability, long-term effectiveness, and patient choice after extensive counseling, a combination of i.v. methylprednisolone and mycophenolate sodium is recommended as first-line treatment. A cumulative dose of 4.5 g of i.v. methylprednisolone in 12 weekly infusions is the optimal regimen. Alternatively, higher cumulative doses not exceeding 8 g can be used as monotherapy in most severe cases and constant/inconstant diplopia. Second-line treatments for moderate-to-severe and active GO include (a) the second course of i.v. methylprednisolone (7.5 g) subsequent to careful ophthalmic and biochemical evaluation, (b) oral prednisone/prednisolone combined with either cyclosporine or azathioprine; (c) orbital radiotherapy combined with oral or i.v. glucocorticoids, (d) teprotumumab; (e) rituximab and (f) tocilizumab. Sight-threatening GO is treated with several high single doses of i.v. methylprednisolone per week and, if unresponsive, with urgent orbital decompression. Rehabilitative surgery (orbital decompression, squint, and eyelid surgery) is indicated for inactive residual GO manifestations.

中文翻译：

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2021 年欧洲格雷夫斯眼眶病组织 (EUGOGO) 格雷夫斯眼眶病医疗管理临床实践指南。

Graves 眼眶病 (GO) 是 Graves 病 (GD) 的主要甲状腺外表现。治疗选择应基于对 GO 临床活动和严重程度的评估。对于大多数 GO 患者而言，早期转诊至专科中心至关重要。危险因素包括吸烟、甲状腺功能障碍、血清促甲状腺激素受体抗体水平高、放射性碘 (RAI) 治疗和高胆固醇血症。在轻度和活动性 GO 中，控制危险因素、局部治疗和硒（缺硒区域）通常就足够了；如果选择 RAI 治疗来管理 GD，则需要低剂量口服泼尼松预防，尤其是在危险因素并存的情况下。对于活动性中重度和威胁视力的 GO，在治疗 Graves 甲状腺功能亢进时，首选抗甲状腺药物。在中度至重度和活动性 GO iv 中，糖皮质激素比口服糖皮质激素更有效且耐受性更好。根据目前的证据和疗效/安全性概况、成本和报销、药物可用性、长期有效性以及广泛咨询后的患者选择，推荐静脉注射甲基强的松龙和霉酚酸钠作为一线治疗。在 12 周输注中累积 4.5 g iv 甲基强的松龙的剂量是最佳方案。或者，在大多数严重病例和恒定/不稳定复视中，可以使用不超过 8 g 的更高累积剂量作为单一疗法。中度至重度和活动性 GO 的二线治疗包括（a）在仔细的眼科和生化评估之后，静脉注射甲基强的松龙（7.5 g）的第二个疗程，(b) 口服泼尼松/泼尼松龙联合环孢素或硫唑嘌呤；(c) 眼眶放疗联合口服或静脉注射糖皮质激素， (d) teprotumumab；(e) 利妥昔单抗和 (f) 托珠单抗。威胁视力的 GO 每周接受数次高单剂量 iv 甲基强的松龙治疗，如果无反应，则进行紧急眼眶减压。康复手术（眼眶减压、斜视和眼睑手术）适用于非活动性残余 GO 表现。