DOI: https://doi.org/10.21203/rs.3.rs-1920698/v1
Background: Vision loss in patients with arteritic anterior ischemic optic neuropathy (A-AION) caused by giant cell arteritis (GCA) is disastrous and GCA is very rare in Asian individuals. We report two Chinese patients presenting with A-AION complicated with cilioretinal artery occlusion (CLAO), and the temporal artery biopsy (TAB) proved the diagnose of GCA.
Case presentation: Two elderly male patients presented with sudden vision loss in the right eyes. Fundus examination of the right eyes revealed chalky, white, swollen optic papilla and swollen region of the retina between the macular and the optic disc. OCT showed a thickened nerve fiber layer of the optic disc and an enhanced signal intensity of the nuclear layer between the macular and the optic disc in OCT. OCTA revealed a decreased density of capillaries around the optic disc and non-perfusion of the deep retina between the macula and the optic disc. The laboratory tests included elevated CRP and ESR. Histopathology of the right temporal artery revealed the infiltration of lymphocytes, plasma cells, histiocytes, and multinucleated giant cells. TAB confirmed the diagnosis of GCA-induced A-AION combined with CLAO. They were treated with intravenous methylprednisolone for 6 days and oral prednisone 1 mg/kg/d, which was gradually tapered. The visual acuities of right eyes were improved in both patients at 6 months follow-up.
Conclusions: GCA can lead to A-AION combined with CLAO in Chinese patients. OCTA is a noninvasive and convenient procedure that can reveal decreased density of capillaries around the optic disc and non-perfusion of the deep retina between the macula and the optic disc through the detected of retinal microcirculation in patient with GCA-induced A-AION combined with CLAO.
Giant cell arteritis (GCA) is a systemic autoimmune disease that mainly affects elderly people. It is characterized by vascular inflammation involving the great and middle arteries [1]. It is very rare in Asian individuals and is more common in White individuals. Arteritic anterior ischemic optic neuropathy (A-AION) is the most common cause of visual dysfunction caused by GCA [2]. In China, most patients with AION have non-arteritic anterior ischemic optic neuropathy (NA-AION) [3]. Here we report two Chinese patients with GCA-induced A-AION combined with cilioretinal artery occlusion (CLAO); in both cases, GCA was confirmed by temporal artery biopsy (TAB).
An 86-year-old male Chinese patient was admitted to hospital 10 hours after experiencing sudden vision loss in his right eye and hallucinations. He had no history of hypertension, hyperlipidemia, diabetes, or new-onset headache. The pulsation of bilateral temporal arteries could not be felt. The best-corrected visual acuity (BCVA) of his right and left eyes were hand motion and 30/50, respectively. A relative afferent pupillary defect (RAPD) and lens opacity were noted in the right eye. A chalky, white, swollen optic papilla on the temporal side, a pale, swollen region of the retina between the macular and infratemporal region of the optic disc, and slightly thin arteries were discovered by fundus examination of the right eye (Fig. 1A). Optical coherence tomography (OCT) revealed a thickened optic disc with high signal intensity and an enhanced signal in the nuclear layer between the macular and optic disc (Fig. 1B). OCT angiography revealed a decreased density of capillaries around the optic disc and an avascular area on the temporal side of the optic disc (Fig. 1C). Magnetic resonance imaging of the right eye revealed a thick optic nerve. Color duplex ultrasonography (CDUS) of the ocular blood flow revealed decreased blood flow through the bilateral central retinal arteries, posterior ciliary arteries, and ophthalmic arteries, as well as thickened walls of the bilateral superficial temporal arteries. Laboratory tests showed decreased erythrocyte (3.68 × 1012/L; normal: 3.8–5.1 ×1012/L) and leukocyte (2.33 × 109/L; normal: 3.5–9.5 × 109/L) counts; normal hemoglobin (116 g/L; normal: 115–150 g/L); very high C-reactive protein (CRP) level (17.1 mg/L; normal: 0–10 mg/L) and erythrocyte sedimentation rate (ESR; 33 mm/h; normal: 0–15 mm/h); and serum negativity for antinuclear antibody (ANA), extractable nuclear antigen (ENA), antineutrophil cytoplasmic antibody (ANCA), rapid plasma regain (RPR) and interferon-γ release assay. Considering the possibility of GCA, methylprednisolone 0.5 g/d was intravenous and right TAB was performed 2 days later. Histopathology revealed a thickened artery wall, a loose outer layer of the artery wall, and the infiltration of lymphocytes, plasma cells, histiocytes, and multinucleated giant cells (Fig. 2). Therefore, he was diagnosed with GCA, A-AION, and CLAO. He was treated with intravenous methylprednisolone at 0.5 g/d for 3 days, 0.25 g/d for the next 3 days and then oral prednisone 1 mg/kg/d, which was gradually tapered. Three months later, his ESR was 6 mm/h and CRP was 3.61 mg/L. The BCVA of his right eye improved to 1/20. CDUS revealed that the blood flow parameters of the posterior ciliary artery were essentially normal in both eyes. The patient was followed for approximately 6 months and he experienced no systemic symptoms.
An 81-year-old male Chinese patient was admitted to our hospital 1 week after a sudden decrease in vision affecting his right eye. Although he was diagnosed with hypertension 5 years earlier, his blood pressure was stably controlled. He had no history of hyperlipemia, diabetes, or headache. His BCVA was 2/20 for his right eye and 10/20 for his left eye. Slit-lamp microscopic examination of his right eye revealed a slightly dilated pupil and RAPD. Fundus examination of the right eye revealed an unclear boundary of the optic disc, a gray–white swollen region on the inferior nasal side, a swollen region of the retina between the macular and the optic disc, and slightly thin arteries (Fig. 3A). OCT revealed a thickened nerve fiber layer of the inferior optic disc (Fig. 3B) and an enhanced signal intensity of the nuclear layer between the macular and the optic disc in OCT (Fig. 3C). OCTA showed non-perfusion of the deep retina between the macula and the optic disc (Fig. 3D), as well as a decreased density of capillaries around the disc (Fig. 3E). His laboratory tests included elevated CRP (35.79 mg/L; normal: 0–10 mg/L) and ESR (65 mm/h; normal: 0–15 mm/h). Computed tomography arteriography revealed mixed plaque deposits on the walls of the bilateral internal carotid arteries, as well as mild stenosis at the beginning of the right internal carotid artery and the right vertebral artery. B-ultrasound revealed significant thickening of the right superficial temporal artery wall. CDUS revealed decreased blood flow of bilateral central retinal arteries, posterior ciliary arteries, and ophthalmic arteries. The right TAB was performed and histopathology showed a thickened vessel wall; infiltration of lymphocytes, plasma cells, and histiocytes, with formation of multinucleated giant cells in connective tissue; and small regions of necrotic-like tissue (Fig. 4). The diagnosis was GCA, A-AION, and CLAO. He was administered intravenous methylprednisolone at a dose of 0.5 g/d for 3 days and 0.25 g/d for 3 days, and then switched to prednisone at a dose of 1 mg/kg/d, which was gradually tapered. One month later, his ESR was 3 mm/h and his CRP was 2.5 mg/L. His BCVA recovered to 20/50 for right eye. The patient was followed for approximately 6 months and experienced no systemic symptoms or worsening visual acuity.
We have reported two rare cases of GCA-induced A-AION combined with CLAO, which were confirmed by TAB, in Chinese patients who experienced a sudden decline in monocular visual acuity. To our knowledge, there are no prior reports of GCA-induced A-AION combined with CLAO in Chinese patients. Timely administration of corticosteroids improved the visual function in both patients and prevented a further decline in visual acuity of the affected eyes, damage of the contralateral eyes, or life-threatening events.
GCA, which is quite rare in Asian individuals, is an autoimmune inflammation that affects large and medium-sized blood vessels and is usually accompanied by systemic symptoms such as headache, scalp tenderness, chewing discomfort, anorexia, fever, and weight loss [4]. GCA may cause sudden loss of visual acuity, with blindness in severe cases. A-AION is the most common cause of permanent vision loss caused by GCA [5]. Vision loss in patients with A-AION is usually disastrous. Most patients have a visual acuity below 20/200, and up to 21% of patients are unable to perceive light. Other causes of blindness in patients with GCA include central retinal artery occlusion (14.8%), CLAO, posterior ischemic optic neuropathy, and ophthalmic artery occlusion [6]. Both of the patients described in our report had GCA, which led to A-AION combined with CLAO. Chalky white swelling of the optic disc and a focal region of gray edema between the macular and optic disc are highly suggestive of tissue ischemia.
Fundus fluorescein angiography (FFA) can be used to diagnose AION and CLAO, which is characterized by a choroidal filling delay [7]. However, as an invasive examination, FFA has some potential risks, including the possibility of allergic reactions to intravenous fluorescein sodium [8]. Unlike FFA, OCTA visualizes the microcirculation by detecting the motion contrast of flowing blood, allowing the ophthalmologist to observe the retinal and choroidal blood supplies in vivo. Furthermore, because OCTA does not require the administration of drugs, it is a safe and noninvasive procedure [9]. OCTA can also track the evolution of the capillary microcirculation in eyes with A-AION. In the acute stage, shallow expansion occurs around capillaries, which is followed by a decrease in the density of capillaries around the optic disc. Loss of perfusion of the paraoptic capillaries results in the loss of the visual field [10]. OCTA has also revealed decreases in the capillary density of the retina and choroid at the side of blood supply in eyes affected with CLAO [11], consistent with the findings in our cases.
Currently, the diagnosis of GCA is based on the American Society of Rheumatology (ACR) criteria published in 1990 [12]: (1) age > 50 years at onset; (2) recent headache; (3) temporal artery disease with tenderness or tenderness of the temporal artery and weak pulsation, except when caused by carotid atherosclerosis; (4) accelerated ESR (> 50 mm/h); and (5) abnormal arterial biopsy findings, such as vasculitis, inflammatory infiltration dominated by monocytes or granulomatous inflammation, often multinucleated giant cells. Although the diagnostic criteria were revised in 2016, with the addition of CRP, fibrinogen, and other parameters, it is still recommended that patients satisfy at least three of the original five criteria for diagnosis [13]. In this report, case 1 satisfied diagnostic criteria (1) and (3), and case 2 satisfied diagnostic criteria (1) and (4). Patients with suspected GCA still require confirmation of diagnosis by TAB.
TAB shows high specificity and is considered the gold standard method for the diagnosis of GCA. Because A-AION usually leads to irreversible visual loss, timely administration of corticosteroid could prevent further visual impairment and reduce the risk of disease recurrence. In order to confirm the diagnosis of GCA and avoid delaying treatment, TAB should be performed as soon as possible, and corticosteroid administration should not be delayed [6].
Limitation of the report is the lack of fluorescence angiography and ICGA results. because the patients were allergic to the contrast medium, thay could not be performed.
In conclusion, GCA can lead to A-AION combined with CLAO. OCTA is a noninvasive and convenient procedure that can reveal decreased density of capillaries around the optic disc and non-perfusion of the deep retina between the macula and the optic disc through the detected of retinal microcirculation in patient with GCA-induced A-AION combined with CLAO. Clinically, TAB and corticosteroid administration should be performed as soon as possible in patients with GCA-induced A-AION combined with CLAO. Corticosteroids can prevent further visual impairment, reduce the risk of disease recurrence and life-threatening accidents.
Giant cell arteritis
Arteritic anterior ischemic optic neuropathy
Cilioretinal artery occlusion
Temporal artery biopsy
Optical coherence tomography
Optical coherence tomography angiography
Best-corrected visual acuity
Relative afferent pupillary defect
C-reactive protein
Erythrocyte sedimentation rate
Antinuclear antibody
Extractable nuclear antigen
Antineutrophil cytoplasmic antibody
Rapid plasma regain
Acknowledgements
The authors thanked the patients who generously agreed to participate in this report.
Authors’ contributions
Zhijian Jiang drafted the manuscript, performed surgery and reviewed the literature. Huiying Ji collected the laboratory materials and assisted in drafting manuscript. Maoli Zhu and Nan Zhang collected and analyzed the data. Qing Chang and Jianhong Dong revised the manuscript and discussions. All authors read and approved the final manuscript.
Funding
No funding was involved in this research.
Availability of data and materials
All datasets used and/or analysed in the current study are available from the corresponding author upon reasonable request.
Ethics approval and consent to participate
Ethics approval for this study was abtained from the Shanghai Xuhui Central Hospital Ethics Committee of Shanghai Xuhui Central Hospital.
Consent for publish
Written informed consent was obtained from the patients for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.
Competing interests
The authors report no conflicts of interest. The authors alone are responsible for the content and the writing of the article.