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Table of Contents
Year : 2023  |  Volume : 5  |  Issue : 1  |  Page : 3

Giant cell arteritis: Is there a link between ocular and systemic involvement?

1 Department of Ophthalmology, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal
2 Department of Pathology, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal

Date of Submission30-Nov-2022
Date of Decision12-Dec-2022
Date of Acceptance14-Dec-2022
Date of Web Publication19-Feb-2023

Correspondence Address:
Mariana G Portela
Rua da Junqueira, 126, 1349-019 Lisbon
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_65_22

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Introduction: Giant cell arteritis (GCA) is a systemic disease that may cause severe visual loss. Our purpose was to determine which factors contribute to ocular manifestations (OMs) in patients with temporal artery biopsy-proven GCA.
Methods: This was a retrospective review of all cases diagnosed in our center from 2010 to 2021 (>6 months follow-up). We analyzed demographic data, comorbidities, type and duration of symptoms before diagnosis, ocular features, and systemic and laboratory findings.
Results: Out of 51 patients (60.8% females, mean age 74.8 ± 13.5), 32 (63%) had OM, of whom 28% had transient visual loss, 69% had permanent visual loss, and 9% had diplopia. Apart from scalp tenderness (P = 0.039), OM did not correlate with other ischemic symptoms such as jaw claudication or headache and were even significantly associated with the absence of fever (P = 0.026). Patients with OM also showed lower values of C-reactive protein (CRP) (P = 0.045).
Conclusion: In our sample, visual involvement was not significantly associated with any systemic symptom apart from scalp tenderness, which highlights the importance of having a high index of suspicion for GCA in an ophthalmology setting, especially in older patients with visual loss. Furthermore, OMs were significantly associated with the absence of fever and lower values of CRP which may suggest a lower inflammatory state may in these patients.

Keywords: Amaurosis fugax, anterior ischemic optic neuropathy, biopsy, giant cell arteritis, temporal arteries

How to cite this article:
Portela MG, Correia MR, Baptista ML, Bruxelas CP, Pinto D, Costa JM. Giant cell arteritis: Is there a link between ocular and systemic involvement?. Pan Am J Ophthalmol 2023;5:3

How to cite this URL:
Portela MG, Correia MR, Baptista ML, Bruxelas CP, Pinto D, Costa JM. Giant cell arteritis: Is there a link between ocular and systemic involvement?. Pan Am J Ophthalmol [serial online] 2023 [cited 2023 May 28];5:3. Available from: https://www.thepajo.org/text.asp?2023/5/1/3/369998

  Introduction Top

Giant cell arteritis (GCA) is the most common idiopathic systemic vasculitis affecting large- and medium-sized arteries in adults over the age of 50 years.[1],[2] The estimated incidence for people over 50 is 3.2 to 5.8/100,000, but it varies according to region, gender, and age.[3],[4] This condition is typically characterized by a systemic presentation, including headache, jaw claudication, scalp tenderness, and constitutional symptoms.[5],[6] However, it may also be accompanied and sometimes preceded by ophthalmologic manifestations such as visual loss.[7],[8] Furthermore, 20% of GCA cases with permanent visual loss do not present with systemic symptoms.[9] When left untreated, it may lead to severe consequences, including visual loss of the other eye or even death.[10],[11],[12] Thereby, the ophthalmologist's prompt recognition of this disease may avoid bilateral ocular involvement and improve general systemic prognosis. A clear connection between ocular and systemic manifestations has not been demonstrated, and if existing, it could help clinicians to be more aware of possible ophthalmologic involvement in some instances.

Our purpose was, therefore, to determine if and which factors contribute to, or are associated with, ocular manifestations (OMs) in patients with temporal artery biopsy (TAB)-proven GCA.

  Methods Top

Study population

This was a comparative retrospective study performed between 2010 and 2021. In collaboration with the pathology department, we collected and reviewed the medical records of all patients diagnosed with a GCA proved by TAB. Patients with a follow-up inferior to 6 months or a GCA diagnosis based on other diagnostic methods were excluded. Two subgroups were then created and compared: patients with GCA with OM and patients with GCA without OM.

Analyzed data

We analyzed the following data: patient demographics (gender, age at diagnosis, and race); previous medical history, including diabetes mellitus, arterial hypertension, obesity, chronic obstructive pulmonary disease, chronic kidney disease, obstructive sleep apnea, thyroid disease, and cardiovascular disease; type and duration of ocular and systemic symptoms before diagnosis; ocular examination at the time of diagnosis and after at least six months of follow-up including best corrected visual acuity (BCVA), slit-lamp and ocular fundus examination, color perception test, intraocular pressure, relative afferent pupillary defect, optical coherence tomography, and visual field testing; and systemic and laboratory findings at the time of diagnosis including body temperature, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and hemoglobin levels, and platelet count.

Systemic manifestations

Systemic symptoms of GCA included headache, localized or diffuse scalp tenderness, jaw or tongue claudication, fatigue, fever (reported by the patient), weight loss, anorexia, and complaints of polymyalgia rheumatica, such as shoulder pain or stiffness, pelvic pain or stiffness, and bilateral aching neck. Body temperature was measured at diagnosis and registered. Fever was defined as an oral or axillary temperature above 37.6°C or an ear temperature above 38.1°C.

Ocular manifestations

The following were considered OMs of GCA: episodes of amaurosis fugax with no other apparent cause, such as carotid or cardioembolic disorders, migraine, occipital seizure, papilledema, optic disc drusen, or intermittent angle-closure glaucoma; visual loss or visual field defects secondary to arteritic anterior ischemic optic neuropathy, posterior ischemic optic neuropathy, central retinal artery occlusion, ocular ischemic syndrome, cilioretinal artery occlusion, or choroidal infarction; and diplopia.

  Results Top

Patient demographics

We included 51 patients diagnosed with a TAB-proven GCA. Among these, 60.8% were females, 92.2% were white, and the mean age was 76.37 ± 8.22 years. Thirty-two patients (62,7%) presented with OMs related to GCA. On average, patients with ocular involvement were 3.53 years older (P = 0.143). In addition, both groups had no difference regarding sex (P = 0.514) or race (P = 0.205). Demographic data are summarized in [Table 1].
Table 1: Demographic data of all patients with giant cell arteritis

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Previous medical history

The previous medical history of all patients is summarized in [Table 2]. Arterial hypertension was the most common systemic comorbidity in both groups, followed by diabetes mellitus and dyslipidemia. There was no significant difference in the prevalence of systemic comorbidities between both groups.
Table 2: Previous medical history of all patients with giant cell arteritis

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Systemic symptoms

Temporal headache was the most common systemic symptom at presentation in both the groups, followed by jaw claudication. Scalp tenderness was reported significantly more frequently by patients with OM (P = 0.028). There was no difference between the two groups in terms of the prevalence of other systemic symptoms. [Table 3] summarizes these findings.
Table 3: Systemic symptoms at presentation of all patients with giant cell arteritis

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The average time between the appearance of the first symptom and the diagnosis of GCA was 22.6 days (median 5.5 days) for patients with OM and 39.8 days (median 30 days) for patients without OM (P = 0.194).

Laboratory and systemic findings

CRP levels were 13.35 mg/mL higher on average in patients without OM (P = 0.045). Other laboratory findings revealed no significant differences. Furthermore, significantly more patients without OM presented with a fever (P = 0.026). [Table 4] compares the laboratory and systemic results of the two groups.
Table 4: Laboratory and systemic findings at diagnosis

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Ocular symptoms and findings

Permanent visual loss was the most common ocular symptom among patients with OM, followed by transient visual loss. Three patients reported diplopia, and three more reported a visual field defect. Most patients with permanent visual loss presented with a BCVA of less than 20/125 (n = 21, 95.5%), and in no case did it improve during follow-up. At the fundoscopic examination, the most common diagnosis was anterior ischemic optic neuropathy (n = 20, 62.5%). Posterior ischemic optic neuropathy was diagnosed in 2 cases, and both initially presented with a normal fundoscopy that evolved to optic atrophy during follow-up. Another three patients had a central retinal artery occlusion, which was diagnosed based on fundoscopic appearance with retinal whitening and a cherry red spot that later progressed to inner retina atrophy. There was no patent cilioretinal artery in any of these cases, and all had a BCVA of less than 20/100. Finally, one patient was identified as having ocular ischemic syndrome. In this case, Doppler ultrasound imaging excluded hemodynamically significant internal carotid artery stenosis. [Table 5] describes the visual symptoms and fundoscopic diagnosis of all patients with OM.
Table 5: Ocular symptoms and signs at fundoscopy

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Six patients had bilateral OM (19%) either with an initial bilateral presentation or a documented sequential bilateral involvement (n = 2). In all cases of initial bilateral presentation, the fundoscopic examination revealed that one eye had been affected first, presenting in most cases with an already established optic nerve atrophy.

Systemic corticosteroid therapy was administered to all patients.

  Discussion Top

Our study identified 51 patients with GCA, proved by a positive TAB. Among these, 32 individuals (63%) presented with OM. Patients without OM were younger, which is consistent with the literature.[13],[14] The influence of sex, however, is still contradictory. In our sample, we did not find significant differences between both groups.[13] Male sex has, nevertheless, been proposed as both a risk factor 15 and a protective factor for cranial ischemic complications in GCA.[15]

The link between OM and systemic diseases in GCA has yet to be established. All of the systemic disorders we investigated had no increased prevalence in any group, including diseases associated with increased cardiovascular risk, such as arterial hypertension, diabetes, and dyslipidemia.

In our work, patients with OMs were more likely to complain of scalp tenderness (P = 0.028). Other systemic symptoms have been associated with higher probability of ocular involvement such as headache[13] and our sample demonstrated a similar trend. Other constitutional symptoms were not significantly different between groups, supporting the need to suspect possible ocular involvement in all cases of GCA.

Patients without OM had significantly higher levels of CRP (P = 0.045) and a greater chance of having a fever (P = 0.026). High CRP level has been associated with a reduced risk of permanent visual loss in other works.[16],[17] The absence of fever has also been associated with an increased risk of cranial ischemic complications.[18] The association of lower inflammatory markers with the absence of OM may lie within the tendency of patients without OM being younger, thus having more ability to mount an inflammatory response. In the sample from Hayreh et al.,[13] patients without OMs had higher levels of ESR, which the same reason may explain.

We also found that patients with OM sought medical care faster. This is probably due to the panic felt by patients with vision loss. In future studies, it could be interesting to understand if patients with OM have a better systemic prognosis.

In our sample, the permanent visual loss was mainly caused by anterior ischemic optic neuropathy. This is still a significant cause of visual loss in GCA.[15] Despite systemic corticosteroid therapy, no patient improved their BCVA during follow-up. However, we believe that high-dose corticosteroids should be urgently started when the suspicion of GCA arises without waiting for the TAB results. Furthermore, nine patients presented with transient visual loss, and six patients demonstrated a normal retina and optic nerve at fundoscopy, although later having a positive TAB. Cases such as these support that amaurosis fugax is a threatening symptom commonly associated with GCA and often progresses to permanent visual loss.[15],[19]

Furthermore, TAB is still considered the gold standard for diagnosing GCA.[20] However, other diagnostic methods, such as Doppler ultrasonography of the temporal artery, magnetic resonance, computed tomography angiography, and 18F-fluorodeoxyglucose positron-emission tomography, may be helpful in the diagnosis of large-vessel vasculitides, such as GCA.[21],[22],[23] By choosing to exclude patients that had not been submitted to TAB, we probably failed to include some individuals that had had the disease. Furthermore, there is evidence in previous studies that the likelihood of receiving a positive TAB is higher in patients with OM, which could also bias our results.[24],[25]

To conclude, GCA remains an essential diagnosis in the ophthalmology clinic with devastating symptoms and signs and often with a poor prognosis. The ophthalmologist must have a high index of suspicion for this entity, even in the absence of characteristic systemic features. OMs of GCA vary widely and may associate with misleading findings such as lower values of CRP and the absence of fever. Raising awareness for this disorder among ophthalmologists is decisive.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

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Liozon E, Dalmay F, Lalloue F, Gondran G, Bezanahary H, Fauchais AL, et al. Risk factors for permanent visual loss in biopsy-proven giant cell arteritis: A study of 339 patients. J Rheumatol 2016;43:1393-9.  Back to cited text no. 17
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  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]


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