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Orbital diseases mimicking graves' orbitopathy: a long-standing challenge in differential diagnosis.
Journal of Endocrinological Investigation ( IF 5.4 ) Pub Date : null , DOI: 10.1007/s40618-019-01141-3
M Marinò 1 , I Ionni 1 , G Lanzolla 1 , A Sframeli 2 , F Latrofa 1 , R Rocchi 1 , C Marcocci 1
Affiliation  

Graves' orbitopathy (GO) is the most common cause of orbital tissue inflammation, accounting for ~ 60% of all orbital inflammatory conditions in the population aged 21-60 years, and for ~ 40% in the population aged > 60 year. GO is observed in 25-30% of patients with Graves' hyperthyroidism and more rarely in association with hypothyroid autoimmune thyroiditis. In addition, a small proportion of GO patients (1-2%) do not have a clinically overt thyroid dysfunction. Clinically, GO is characterized by proptosis, inflammation involving the eyelids and the conjunctiva, extraocular muscle hypertrophy, with consequent reduction of ocular motility and diplopia, and in the most severe cases, compression of the optic nerves at the orbital apex, with reduction of visual acuity. At CT scan or MRI, a muscle increase involving the superior, medial and inferior rectus is quite typical. In the most severe forms, compression of the optic nerves at the orbital apex can be observed. Euthyroid GO is usually an early sign of a full-blown Graves' disease; however, in some cases, the orbital disease can remain isolated. Moreover, euthyroid GO can rarely be unilateral, which makes the picture even more confusing. Under those circumstances, the diagnostic process becomes obviously quite difficult, having other conditions mimicking GO been excluded. A number of inflammatory conditions affecting orbital tissue can mimic GO, thereby requiring an accurate evaluation for a proper differential diagnosis. The majority of these conditions are immune mediated. Most of them are benign, but they can be rather aggressive and some can cause visual loss. The most common inflammatory condition affecting orbital tissues and mimicking GO is idiopathic orbital inflammation. Other, more rare, orbital diseases that should be considered in the differential diagnosis are infections, orbital manifestations of systemic diseases, primitive and secondary orbital neoplasms, and orbital vascular alterations. In most instances, when an orbitopathy occurs in the absence of hyperthyroidism, the diagnosis of the disease underlying the ocular symptoms and signs is based on exclusion of the other conditions. Here we review the conditions that can mimic GO and how to distinguish them from this obnoxious eye disease.

中文翻译:

模仿格雷夫斯眼眶病的眼眶疾病:鉴别诊断中的长期挑战。

格雷夫斯眼眶病 (GO) 是眼眶组织炎症的最常见原因,占 21-60 岁人群中所有眼眶炎症状况的约 60%,占 60 岁以上人群的约 40%。在 25-30% 的 Graves 甲状腺功能亢进患者中观察到 GO,与甲状腺功能减退性自身免疫性甲状腺炎相关的情况更少。此外,一小部分 GO 患者(1-2%)没有临床上明显的甲状腺功能障碍。临床上,GO的特点是眼球突出、眼睑和结膜发炎、眼外肌肥大,导致眼球运动能力下降和复视,最严重的情况下,眶尖处的视神经受压,视力下降敏锐度。在 CT 扫描或 MRI 中,涉及上肢的肌肉增加,内直肌和下直肌比较典型。在最严重的形式中,可以观察到眶尖处的视神经受压。甲状腺功能正常的 GO 通常是 Graves 病成熟的早期征兆。然而,在某些情况下,眼眶疾病可以保持孤立。此外,甲状腺功能正常的 GO 很少是单侧的,这使得图片更加混乱。在这种情况下,诊断过程显然变得相当困难,排除了其他模仿 GO 的情况。许多影响眼眶组织的炎症状况可以模仿 GO,因此需要准确评估以进行正确的鉴别诊断。这些病症中的大多数是免疫介导的。它们中的大多数是良性的,但它们可能相当具有攻击性,有些会导致视力丧失。影响眼眶组织和模仿 GO 的最常见炎症是特发性眼眶炎症。在鉴别诊断中应考虑的其他更罕见的眼眶疾病包括感染、系统性疾病的眼眶表现、原始和继发性眼眶肿瘤以及眼眶血管改变。在大多数情况下,如果在没有甲状腺功能亢进的情况下发生眼眶病,则基于眼部症状和体征的疾病的诊断是基于排除其他疾病。在这里,我们回顾了可以模仿 GO 的条件以及如何将它们与这种令人讨厌的眼病区分开来。系统性疾病、原发性和继发性眼眶肿瘤以及眼眶血管改变的眼眶表现。在大多数情况下,如果在没有甲状腺功能亢进的情况下发生眼眶病,则基于眼部症状和体征的疾病的诊断是基于排除其他疾病。在这里,我们回顾了可以模仿 GO 的条件以及如何将它们与这种令人讨厌的眼病区分开来。系统性疾病、原发性和继发性眼眶肿瘤以及眼眶血管改变的眼眶表现。在大多数情况下,如果在没有甲状腺功能亢进的情况下发生眼眶病,则基于眼部症状和体征的疾病的诊断是基于排除其他疾病。在这里,我们回顾了可以模仿 GO 的条件以及如何将它们与这种令人讨厌的眼病区分开来。
更新日期:2020-03-12
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