Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
Department of Ophthalmology and Vision Sciences, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
J Neurol Sci. 2022 May 15;436:120222. doi: 10.1016/j.jns.2022.120222. Epub 2022 Mar 6.
Diagnosing giant cell arteritis (GCA) can be challenging due to the variable clinical presentation and lack of systemic symptoms in approximately 20% of cases, which are termed occult GCA. GCA affects the ocular circulation in more than half of cases and can cause irreversible bilateral blindness if not rapidly recognized and treated. We compared clinical and laboratory findings of patients with occult and systemic temporal artery biopsy (TAB)-positive GCA with ocular manifestations.
Retrospective chart review of patients with TAB-positive GCA with ocular manifestations seen at a tertiary neuro-ophthalmology practice between 2015 and 2020.
GCA was occult in 14.3% (6/42) and systemic in 85.7% (36/42) of patients. Occult GCA affected older patients (average age 84.6 ± 5.3 vs 75.9 ± 8.2). There was no significant difference in ESR or CRP levels between two cohorts. In patients with occult GCA, 83.3% (5/6) presented with AION, and 16.7% (1/6) with isolated cranial nerve six palsy (CN6P). In the systemic GCA cohort, 47.2% (17) presented with AION, 5.6% (2) with CRAO, 2.8% (1) with PION, 2.8% (1) with isolated CWS, 2.8% (1) had both CRAO and AION, 2.8% (1) isolated cranial nerve 4 palsy (CN4P), 2.8% (1) isolated CN6P, 22.2% (8) had history of amaurosis fugax but normal examination, and 11.1% (4) had visual symptoms but a normal exam. Three of the above patients with AION also had CN4P. Of patients diagnosed with AION, presenting visual acuity in the affected eye(s) was significantly worse in patients with occult GCA. Involvement of multiple ocular circulations was seen in six patients in the systemic group but none in the occult group.
Occult GCA occurs more commonly in older patients who most of the time present with AION. Levels of inflammatory markers were similar in the occult and systemic groups. Though ocular ischemic manifestations were similar in both cohorts, in systemic GCA involvement of multiple ocular circulations is likely more common GCA should always be considered in older patients who present with signs of retinal and/or optic nerve head ischemia (AION and/or CWS) and new onset of CNPs, especially in patients where more than one ocular arterial circulation is involved.
由于大约 20%的病例临床表现不典型且缺乏全身症状，因此诊断巨细胞动脉炎（GCA）具有挑战性，这些病例被称为隐匿性 GCA。GCA 影响超过一半病例的眼部循环，如果不能迅速识别和治疗，可能导致双侧失明。我们比较了有眼部表现的隐匿性和系统性颞动脉活检（TAB）阳性 GCA 患者的临床和实验室检查结果。
回顾性分析 2015 年至 2020 年在一家三级神经眼科诊所就诊的 TAB 阳性 GCA 伴眼部表现的患者的病历。
GCA 在 14.3%（6/42）的患者中为隐匿性，在 85.7%（36/42）的患者中为系统性。隐匿性 GCA 影响年龄较大的患者（平均年龄 84.6±5.3 岁 vs 75.9±8.2 岁）。两组患者的 ESR 或 CRP 水平无显著差异。在隐匿性 GCA 患者中，83.3%（5/6）表现为 AION，16.7%（1/6）为单纯 CN6P。在系统性 GCA 组中，47.2%（17）表现为 AION，5.6%（2）为 CRAO，2.8%（1）为 PION，2.8%（1）为单纯 CWS，2.8%（1）为同时发生 CRAO 和 AION，2.8%（1）为单纯 CN4P，2.8%（1）为单纯 CN6P，22.2%（8）有短暂性黑矇史但检查正常，11.1%（4）有视觉症状但检查正常。上述 3 例 AION 患者还伴有 CN4P。在诊断为 AION 的患者中，隐匿性 GCA 患者患眼视力明显更差。在系统性组中有 6 名患者出现多种眼部循环受累，而隐匿性组中无一例。
隐匿性 GCA 更常见于年龄较大的患者，这些患者多数时候表现为 AION。隐匿性和系统性 GCA 组的炎症标志物水平相似。尽管两组的眼部缺血表现相似，但在系统性 GCA 中，多个眼部循环受累可能更常见。对于出现视网膜和/或视神经乳头缺血（AION 和/或 CWS）和新发性 CNPs 的老年患者，尤其是在涉及多条眼动脉循环的患者，应始终考虑 GCA 的可能性。
