Giant Cell Arteritis Presenting as Bilateral Optic Perineuritis in an African Man

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Gour Wang, MD Giant Cell Arteritis Presenting as Bilateral Optic Perineuritis in an African Man Seema Emami, MD, David Howarth, MD, Edward Margolin, MD O ptic perineuritis denotes inflammation of the optic nerve sheath with sparing of axons and is usually associated with infectious and inflammatory etiologies. We present a rare case of biopsy-proven giant cell arteritis (GCA) causing bilateral optic perineuritis and inflammation of multiple branches of descending aorta raising the possibility that Takayasu arteritis (TA) and GCA are the spectrum of the same disease. A 60-year-old healthy Eritrean-Canadian man developed left-sided headache. He was seen by his family physician, empirically diagnosed with sinusitis and completed 2 courses of oral antibiotics without improvement. One month later, he noticed blurred vision in the right eye. He was also fatigued, lost some weight, and noticed that chewing became painful for him. On examination, central acuity was 20/20 in each eye, there was mild right relative afferent pupillary defect, and right optic nerve head appeared mildly elevated with several peripapillary cotton wool spots (Fig. 1A). Humphrey visual fields (24-2 algorithm) demonstrated right inferior altitudinal defect (Fig. 1B). Erythrocyte sedimentation rate was 128 mm/h and C-reactive protein 91 mg/L (normal less than 9) with a relative thrombocytosis of 484,000 cells/mL. Empiric treatment with threeday course of intravenous (IV) methylprednisolone 1 g daily commenced. MRI of the brain and orbits demonstrated bilateral intraorbital optic nerve sheath enhancement with mild orbital fat stranding (Fig. 2A) with sparing of the optic nerve axons. Computed tomography (CT) of the abdomen revealed circumferential wall thickening of the infrarenal abdominal aorta suspicious for vasculitis. CT angiography of the chest further confirmed segmental thickening of the descending thoracic aorta and unilateral proximal superior femoral artery, as well as circumferential thickening of the infrarenal aorta and both common iliac arteries (Fig. 2B). Inflammatory and infectious Department of Ophthalmology and Medicine, University of Toronto, Toronto, Canada. The authors report no conflicts of interest. Address correspondence to Edward Margolin, MD, University of Toronto, Department of Ophthalmology and Medicine, 801 Eglinton Avenue West, Suite 301, Toronto, ON M5N 1E3, Canada; E-mail: [e-mail] Emami et al: J Neuro-Ophthalmol 2021; 41: e149-e152 screen including ANA, ANCA, HIV, syphilis, and hepatitis C virus serologies were unrevealing. A right temporal artery biopsy revealed active granulomatous arteritis with inflammation present mainly in the media with some minor spillover into the intima and adventitia. Disruption of the internal elastic lamina by the inflammatory process was noted. After treatment with IV prednisone, patient was switched to oral prednisone 60 mg daily as well as methotrexate 25 mg daily. When reassessed 3 months after the initial presentation, visual acuity remained stable and all systemic symptoms were resolved. DISCUSSION Enhancement of the optic nerve sheath with sparing of the optic nerve axons, representing optic perineuritis, is exceedingly rare in GCA and has not been previously reported in non-Caucasian individuals (1–4). Although involvement of aortic arch can be seen in up to 80% of patients with GCA on CT angiography and positron emission tomography, stenosis and inflammation of the descending aorta is rare and is usually considered more compatible with other largevessel vasculitides, namely TA (5). Optic perineuritis likely represents inflammation of the small pial arteries that circumferentially supply the entire orbital portion of the optic nerve and usually occurs in idiopathic orbital inflammatory disease as well as systemic inflammatory or infectious diseases (6). As the optic nerve axons themselves are typically spared, central vision is usually preserved in optic perineuritis. In severe cases, vision loss may develop secondary to nerve sheath thickening and subsequent ischemia of the axons (6). By contrast, vision loss in GCA is typically due to vasculitic occlusion of the relatively larger posterior ciliary arteries and/or central retinal artery. In our case, radiographic appearance of perineural inflammation was accompanied by a swollen right optic nerve head with corresponding altitudinal visual field defect, the latter reflecting involvement of the short posterior ciliary arteries. This suggests vasculitis affecting both small- and medium-sized vessels in this typically medium- and largevessel disease. Our patient demonstrated extensive large vessel disease as well with descending aortitis to the level of the infrarenal aorta and extension into the common iliac arteries. The e149 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 1. A. Formal visual field testing (Humphrey 24-2 algorithm) was performed with good reliability parameters. Testing revealed an inferior altitudinal defect in the right eye and an early arcuate defect in the left eye. Visual field defects resolved substantially after steroid therapy. B. Fundus examination showed elevation of the right eye optic nerve head with peripapillary cotton wool spots, suggestive of intraretinal ischemic changes. Right eye optic nerve head elevation was confirmed on optical coherence tomography, which further showed thickened retinal nerve fiber layer in the right eye. degree of large-vessel involvement in this case highlights the broad overlap between GCA and TA. Both diseases are known to cause granulomatous aortic inflammation; however, aortitis is a hallmark of TA while GCA has a predilection for intracranial vessels (5). Conversely, GCA patients with large-vessel involvement are less likely to present with cranial symptoms (5,7). There is a growing understanding, however, that TA and GCA represent a spectrum of the same disease rather than 2 distinct entities (7–11). Histologically, GCA and Takayasu arteritis are undistinguishable from each other, and the differentiating between these 2 entities is only possible within clinical context (8). Differences in the age of onset, race predilection, and pat- e150 tern of large-vessel inflammation have been challenged by reports of patients who meet the criteria for both diseases (7,10,11). Our case supports this argument: Intracranial symptoms were relatively mild, inflammatory changes were demonstrated across small, medium, and large vessels, and the burden of large-vessel disease was significant. Patient’s relatively young age and African ethnicity also hint more toward late-onset TA (which most often occurs in patients younger than 40 years of age) rather than GCA which is rare in non-Caucasians. This is an unusual case of optic perineuritis secondary to GCA in an African patient. The presentation further demonstrates the similarities between GCA and TA, calling Emami et al: J Neuro-Ophthalmol 2021; 41: e149-e152 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 2. A. Gadolinium-enhanced MRI of the brain and orbits showed enhancement of the bilateral intraorbital optic nerve sheaths and mild fat-stranding, as seen on these T1-weighted axial (left) and coronal (right) images. B. Computed tomography (CT) and CT angiography demonstrated thickening of multiple large-caliber vessels in the thorax and abdomen, including the inferior abdominal aorta (left panel), descending thoracic aorta (center panel) and infrarenal aorta, and common iliac arteries (right panel). into question the divide between these vasculitic entities. Recognizing GCA and TA as a disease spectrum rather than as distinct diagnoses would facilitate earlier diagnosis of atypical cases of large-vessel vasculitis (10). This case highlights the potential for atypical small-vessel involvement in GCA manifesting radiologically as optic perineuritis and is a reminder that clinicians should maintain a low index of suspicion for vision-threatening inflammatory disease even in atypical cases. It highlights the importance of large-vessel imaging in patients with GCA and the potential overlap between GCA and TA. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: E. Margolin, S. Emami, and D. Howarth; b. Acquisition of data: E. Margolin and S. Emami; c. Analysis and interpretation of data: D. Howarth, E. Margolin, and S. Emami. Category 2: a. Drafting the manuscript: E. Margolin and S. Emami et al: J Neuro-Ophthalmol 2021; 41: e149-e152 Emami; b. Revising it for intellectual content: E. Margolin, D. Howarth, and S. Emami. Category 3: a. Final approval of the completed manuscript: E. Margolin, D. Howarth, and S. Emami. REFERENCES 1. Liu TY, Miller NR. Giant cell arteritis presenting as unilateral anterior ischemic optic neuropathy associated with bilateral optic nerve sheath enhancement on magnetic resonance imaging. J Neuroophthalmol. 2015;35:360–363. 2. Morgenstern KE, Ellis BD, Schochet SS, Linberg JV. 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