Giant cell arteritis (GCA) is a systemic vasculitis of unknown etiology commonly involving cranial arteries. Although arteritic ischemic optic neuropathy represents the most common sight-threatening manifestation of GCA, retinal and choroidal ischemia are also previously described associations.1,2 Urgent diagnosis based on history and clinical exam, lab testing for elevation of systemic inflammatory markers, and, ultimately, superficial temporal artery biopsy displaying intimal thickening and giant cell formation is essential, as prompt initiation of corticosteroids may prevent ischemia-related vision loss, especially in the contralateral unaffected eye.
A 64-year-old man was referred for evaluation of painless sequential bilateral vision loss. He reported sudden onset right eye vision loss of 3 weeks' duration. Two weeks prior to evaluation, he awoke with diplopia, vomiting, headache, and unsteady gait. An MRI at an outside hospital revealed a small right pontine stroke. He was started on aspirin with resolution of diplopia but unchanged blurred vision in his right eye. No ophthalmologic evaluation was performed at that time. During the next 2 weeks he had continued headache, with additional symptoms of scalp tenderness, jaw claudication, myalgias, and generalized fatigue. Two days prior to presentation, he noted painless decreased left eye vision, and he presented to us when his vision declined to the level that he could no longer read.
On examination, visual acuity (VA) was 20/300 in the right eye (OD) and 20/400 in the left eye (OS). Pupils were reactive without relative afferent pupillary defect. Intraocular pressures and anterior segment examination were unremarkable without anterior chamber inflammation. Dilated examination revealed quiet vitreous and pink, distinct optic nerve heads bilaterally. There was a subfoveal geographic area of pigmentary disruption in the macula OD and a placoid area of subfoveal whitening OS (Figure 1). Spectral-domain optical coherence tomography (SD-OCT) OD revealed foveal thinning with outer retinal atrophy and irregular thickening of the retinal pigment epithelium (RPE), whereas OS displayed loss of the ellipsoid zone, irregular disruption of the external limiting membrane, and hyperreflectivity of outer nuclear layer (Figure 2). Fluorescein angiography (FA) OS showed delayed filling of the macular choroid and late staining of the placoid lesion. Indocyanine green angiography (ICGA) showed hypofluorescence of the lesions persisting throughout the study but patchy filling of large choroidal vessels (Figure 3).
Color fundus photo showing bilateral placoid maculopathy at presentation.
(A) Spectral-domain optical coherence tomography (SD-OCT) at presentation through the subacute placoid lesion of the right eye shows foveal thinning with loss of the outer retinal layers and irregular thickening of the retinal pigment epithelium. (B) SD-OCT at presentation through the acute placoid lesion of the left eye shows outer nuclear layer hyperreflectivity, loss of the ellipsoid band, and a subfoveal hyporeflective space.
(A) Fluorescein angiogram (FA) in the early venous phase, left eye shows diffuse macular hypofluorescence consistent with choroidal nonperfusion but regular filling of the retinal vasculature. (B, C) Mid- and late-phase FA, right eye displaying prominent staining especially of the borders of the placoid lesion. Two smaller satellite lesions (white arrows) are also evident. (D, E) Mid- and late-phase FA, left eye displaying staining of the body of the placoid lesion, consistent with a more acute process. (F, G) Mid- and late-phase indocyanine green angiogram (ICGA), right eye displaying hypofluorescence of the placoid lesion as well as the smaller satellite lesions. Careful observation reveals that the area of hypofluorescence is slightly larger than the area of the placoid lesion, indicating that blockage from overlying retinal pigment epithelium damage does not fully explain the observed choroidal nonfilling. (H, I) Mid- and late-phase ICGA, left eye displays hypofluorescence of the placoid lesion although there is filling of some of the large choroidal vessels (yellow arrow).
Based on the constellation of symptoms, the patient was started on high-dose intravenous corticosteroids due to concern for possible GCA. Subsequent laboratory testing revealed elevated ESR (59 mm/hr) and CRP (14 mg/L), and temporal artery biopsy was consistent with GCA bilaterally. The patient reported almost immediate resolution of headache and jaw claudication with initiation of steroids with some visual improvement OS (20/60) more than OD (20/200). He was eventually switched to mycophenolate for long term immunosuppression. VA has gradually decreased to counting fingers OD and 20/400 OS with 30 months of follow-up, with severe photoreceptor and RPE atrophy seen on follow-up imaging (Figure 4).
Color fundus photo (A, B) at 30 months post-presentation showing central retinal pigment epithelium (RPE) atrophy and irregular pigmentary clumping. Spectral-domain optical coherence tomography (C, D) reveals outer retinal and RPE atrophy in both eyes with loss of the ellipsoid band. There is hyperreflective material in the subretinal space in the left eye (D) more than the right eye (C). Enhanced depth imaging reveals preservation of the large choroidal vessels but choriocapillaris loss.
Ischemic optic neuropathy is the most common cause of vision loss associated with GCA, but there are several previous reports of GCA-related choroidal ischemia.2,3 Although most of these case reports describe generalized or multifocal choroidal ischemia, Olali et al. have reported a case of focal subfoveal unilateral ischemia similar to our case.4 Their report, however, did not include OCT, fundus autofluorescence, or ICGA imaging, nor did it have long-term follow-up.
In 1990, Hayreh published a FA description of the in vivo anatomy of the choroidal circulation. In this paper he described “watershed zones,” areas particularly vulnerable to ischemia due to their location at the border zone of perfusion of more than one posterior ciliary artery.5 The submacular region represents the watershed zone of the temporal short posterior ciliary arteries.6 More recently, OCT angiography (OCTA) has revealed choriocapillaris hypoperfusion as a component of placoid maculopathies, including both persistent placoid maculopathy and acute posterior multifocal placoid pigment epitheliopathy.7,8 We propose that in this unusual presentation of GCA, temporal short posterior ciliary arteritis resulted in compromise of the subfoveal choriocapillaris and subsequent ischemia-mediated damage to the overlying RPE and outer retina in a placoid pattern. Interestingly, ICGA in our case does show some preservation of the large choroidal vessels in this location, indicating that even incomplete occlusion of temporal short posterior ciliary arteries was sufficient to cause hypoperfusion in the watershed zone of the subfoveal choriocapillaris.
The asymmetric appearance in our case is presumably due to the asynchronous involvement of the two eyes, with OD involvement preceding OS by approximately 2 weeks, and is consistent with the often sequential ocular involvement previously reported in GCA. Multimodal imaging at presentation already demonstrates outer retinal atrophy OD due to more chronic ischemic damage, whereas the more acutely involved OS displays outer nuclear layer hyperreflectivity consistent with the retinal opacification seen on clinical exam. Long-term follow-up demonstrates RPE and outer retinal atrophy in both eyes with resulting vision loss.
In summary, choroidal ischemia is an unusual but sight-threatening potential manifestation of giant cell arteritis. GCA should be considered in the differential diagnosis of placoid maculopathy, as early diagnosis and treatment can potentially prevent further ocular ischemic complications or even potentially fatal consequences of systemic vasculitis.
- Melberg NS, Grand MG, Dieckert JP, et al. Cotton-wool spots and the early diagnosis of giant cell arteritis. Ophthalmology. 1995;102(11):1611–1614. doi:10.1016/S0161-6420(95)30820-2 [CrossRef]
- Quillen DA, Cantore WA, Schwartz SR, Brod RD, Sassani JW. Choroidal nonperfusion in giant cell arteritis. Am J Ophthalmol. 1993;116(2):171–175. doi:10.1016/S0002-9394(14)71281-4 [CrossRef]
- Cohen S, Gardner F. Bilateral choroidal ischemia in giant cell arteritis. Arch Ophthalmol. 2006;124(6):922. doi:10.1001/archopht.124.6.922 [CrossRef]
- Olali C, Aggarwal S, Ahmed S, et al. Giant cell arteritis presenting as macular choroidal ischaemia. Eye (Lond). 2011;25(1):121–123. doi:10.1038/eye.2010.169 [CrossRef]
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- Puche N, Hera R, Terrada C, et al. Persistent placoid maculopathy imaged by optical coherence tomography angiography. Retina Cases Brief Rep. 2016;10(4):297–301.
- Heiferman MJ, Rahmani S, Jampol LM, et al. Acute posterior multifocal placoid pigment epitheliopathy on optical coherence tomography angiography. Retina. 2017Nov;37(11):2084–2094. doi:10.1097/IAE.0000000000001487 [CrossRef]