Choroidal ischemia as one cardinal sign in giant cell arteritis

Casella et al. International Journal of Retina and Vitreous https://doi.org/10.1186/s40942-022-00422-z (2022) 8:69 International Journal of Retina and Vitreous Open Access ORIGINAL ARTICLE Choroidal ischemia as one cardinal sign in giant cell arteritis Antonio M. B. Casella1* , Ahmad M. Mansour2, Souza EC3, Rodrigo B. do Prado1, Rodrigo Meirelles4, Keye Wong5, Salma Yassine6 and Mário Luiz R. Monteiro3 Abstract Purpose: To describe chorioretinal signs in a case series of Giant Cell Arteritis (GCA). Methods: This is a multicenter retrospective observational case series with GCA that presented with a headache and an abrupt, unilateral loss in vision. Workup included temporal artery biopsies, intravenous fluorescein angiography, optical coherence tomography (OCT), optical coherence tomography angiography (OCTA), blood levels of erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). Results: There are a total of 8 GCA instances presented. Average age was 74.5. (Range 68–83 years). The patients reported that one eye’s visual loss had suddenly started, along with a fresh headache and other systemic symptoms. Eight patients exhibited choroidal ischemia, five paracentral acute middle maculopathy (PAMM) lesions, five cotton wool spots, four anterior ischemic optic neuropathy, and one central retinal arterial occlusion at the time of presentation. The average ESR at presentation was 68 mm/hr (range 4–110), and 4/6 individuals had a significant increase. The mean CRP level was 6.2 mg/dL (range 2.0–15.4), and the level was always over the normal range. All patients’ temporal artery biopsies were positive. Conclusion: Alongside PAMM lesions, cotton wool spots, anterior ischemic optic neuropathy, and central retinal artery occlusion, choroidal ischemia is a key angiographic indicator in the diagnosis of GCA. It may be crucial to recognize these typical ischemic chorioretinal signs while diagnosing GCA. Keywords: Giant cell arteritis, Choroidal hypoperfusion, Paracentral acute middle maculopathy, Fluorescein angiography, OCTA, Arteritic anterior ischemic optic neuropathy, Cotton-wool spots Introduction Giant cell arteritis (GCA) is a medium to large vessel granulomatous vasculitis of autoimmune etiology with predilection to the cranial branches of the aortic artery [1–32] GCA has multisystem manifestations (new onset temporal headache, jaw claudication, low grade fever), propensity to the elderly population with an average age of onset of 75 years, and a strong female predominance *Correspondence: 1 Department of Surgery, Health Sciences Center, Londrina State University, 60 Robert Koch Av 86038, Londrina, Paraná, Brazil Full list of author information is available at the end of the article [1, 2]. Since the involvement of the contralateral eye can increase to 60% when left untreated [8], visual loss is the most feared and irreversible complication of GCA, and therapy with a high-dose corticosteroid (and most recently tocilizumab) lowers the incidence of blindness. Vision loss results from either central retinal artery occlusion (CRAO) or posterior ciliary artery (PCA) occlusion manifesting as arteritic anterior ischemic optic neuropathy (A-AION) [10, 12]. The only way to diagnose many ischemic events occurring outside of the papillomacular area is with intravenous fluorescein angiography, indocyanine angiography or optical coherence tomography angiography (OCTA). These events can involve the choroid (choroidal ischemia) [11, 12] or the retina © The Author(s) 2022. 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The Creative Commons Public Domain Dedication waiver (http://creativeco mmons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. F F F F F F M M 1 2 3 4 5 6 7 8 83 71 79 72 68 75 78 70 Age Visual loss OS Visual loss OS Visual loss OS Visual loss OS Visual loss OS loss OS Visual Visual loss OS Visual loss OS Visual Com- plaint Yes Yes Yes Yes Yes Yes Yes Yes 20/25 20/20 20/30 20/20 20/70 20/50 CF 20/20 SysteInitial VA OD mic Signs 20/40 20/20 CF 20/800 20/20 20/30 20/80 CF Initial VA OS 20/25 20/20 20/30 20/20 20/25 20/25 20/60 20/20 Final VA OD 20/40 20/800 20/500 20/200 20/20 20/20 20/25 20/200 Final VA OS 6 3 3 36 36 12 60 48 FU 48/2.8 59/2.9 92/15.4 4/2.0 110/7.5 60/10.4 74/2.5 99/6.3 ESR/CRP OS OS OS OU OD OU OU OU Choroi- dal Ischemia No No No No No No No OD CRAO No No OS No OU OU OU OD PAMM No No OS OU No OS OU OU CWS OS OS OS No OD later OD OU OD A-AION F female, M male, OD oculus dexter, OS oculus sinister, OU oculus uterque, VA best spectacle corrected visual acuity, CF counter fingers, ESR erythrocyte sedimentation rate (mm/hr), CRP C-reactive protein (mg/dL), PAMM paracentral acute middle maculopathy, CWS cotton-wool spots, A-AION arteritic anterior ischemic optic neuropathy, CRAO central retinal artery occlusion, FU follow up (months) Sex Case Table 1 Demographics, clinical complaints, initial and final visual acuity and multimodal analysis findings in 8 patients with GCA Casella et al. International Journal of Retina and Vitreous (2022) 8:69 Page 2 of 11 Casella et al. International Journal of Retina and Vitreous (2022) 8:69 Page 3 of 11 (cotton-wool spots (CWS) and paracentral acute middle maculopathy (PAMM) [13–16]. The purpose of this study is to describe such circulatory ischemic events in a case series of GCA using multimodal imaging of the choroidal and retinal circulation. sinister) (range 20/20 to CF both eyes) while mean final visual acuity was 20/27 OD and 20/94 OS (range 20/20 to 20/80 OD; 20/20 to 20/800 OS) with a mean follow-up of 25.5 months (range 3–60). Methods This multicenter, retrospective, observational case study examined the multimodal imaging results for 16 eyes of 8 patients treated for GCA at 6 ophthalmology clinics between January 2013 and December 2020. The descriptive study received ethical committee approval, the researchers agreed to a confidentiality agreement, and informed consent was no (...truncated)