When the Eyes Pave the Way: Takayasu Retinopathy as the First Sign of Takayasu Arteritis

When the Eyes Pave the Way: Takayasu Retinopathy as the First Sign of Takayasu Arteritis | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report When the Eyes Pave the Way: Takayasu Retinopathy as the First Sign of Takayasu Arteritis Vipin Rana, Vikas Sharma, Kunal Kishore, Eram Thakur, Ranjit Goenka, and 2 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7438009/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 24 Oct, 2025 Read the published version in BMC Ophthalmology → Version 1 posted 10 You are reading this latest preprint version Abstract Background Takayasu arteritis (TAK) is a granulomatous large-vessel vasculitis of unknown etiology that predominantly affects the aorta and its branches. Ocular involvement occurs due to ischemia from vascular stenosis or occlusion and may present as transient visual loss, Takayasu retinopathy (TR), or ischemic optic neuropathy. In rare cases, ocular symptoms can be the initial manifestation, underscoring the importance of early recognition. Case Presentation We report a 29-year-old male who presented with progressive blurring of vision, more marked in the left eye, for nine months. Systemic symptoms included presyncope and upper limb claudication. Ocular examination revealed multiple mid-peripheral microaneurysms and a wreath-like type 3 arteriovenous anastomosis near the left optic disc. Fundus fluorescein angiography showed 360° peripheral capillary nonperfusion, while Optical coherence tomography angiography demonstrated capillary dropout in the macular area in left eye. Carotid Doppler and CT angiography revealed diffuse mural thickening and near-total occlusion of major aortic arch branches. Raised C-reactive protein with negative anti-nuclear antibodies supported the diagnosis. Based on these findings, a diagnosis of TAK with stage 4 TR was made. The patient was treated with intravenous corticosteroids followed by immunosuppressants, along with panretinal photocoagulation and intravitreal ranibizumab in the left eye. Conclusion This case highlights decreased vision as the initial and predominant manifestation of TAK, accompanied by systemic vascular symptoms. The presence of advanced TR with type 3 anastomosis reflected severe, chronic ischemia. Recognizing ocular signs in conjunction with systemic features such as claudication and syncope is vital for early diagnosis. Prompt systemic immunosuppression with adjunctive ocular interventions can prevent irreversible visual loss and systemic complications. Takayasu arteritis Takayasu retinopathy Vasculitis Claudication Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Background Takayasu arteritis (TAK) is an inflammatory disease of unknown etiology characterized by granulomatous vasculitis affecting the aorta, its main branches and the arteries. It occurs commonly in women of childbearing age [ 1 ]. The disease progresses through inflammatory stages that can lead to segmental stenosis, occlusion, dilatation, or aneurysm, resulting in a variety of systemic symptoms, such as extremity pain, claudication, bruits, reduced or absent pulses, and alterations in blood pressure. While TAK typically progresses slowly, it can sometimes present acutely with severe complications such as sudden visual loss or stroke [ 2 ]. Ocular manifestations of TAK, which are often due to reduced blood flow from carotid artery occlusion, include transient visual disturbances, anterior segment ischemia, Takayasu retinopathy (TR), and ischemic optic neuropathy. Additionally, renal artery involvement can lead to hypertensive retinopathy [ 3 , 4 ]. This report presents a rare case of TA in which decreased vision was the initial and predominant symptom. This ocular manifestation was accompanied by syncope, unequal blood pressures in both upper limbs and bilateral upper limb claudication. Thus, this case underscores the importance of recognizing ocular symptoms as potential early indicators of this systemic vasculitis, particularly when accompanied by signs of vascular compromise. Case Presentation A 29-year-old male presented to our tertiary care hospital with complaints of progressive blurring of vision in both eyes, which was more pronounced in the left eye, over the past nine months. He also reported episodes of presyncope lasting 8–10 seconds without any loss of consciousness or focal neurological deficits for five months. Additionally, he experienced unilateral pain in the left upper limb, extending from the shoulder to the hand. There was no history of constitutional symptoms such as weakness, fever, or weight loss. On examination, his best-corrected visual acuity was 20/20 in the right eye and 20/50 in the left eye. The patient’s intraocular pressure was 12 mmHg in both eyes. The anterior segment examination of both eyes was unremarkable. Fundus examination revealed multiple microaneurysms in the mid-peripheral retina across all quadrants, along with a wreath-like arteriovenous anastomosis near the left optic disc (Fig. 1 ). Systemic examination revealed absent brachial and radial pulses on both sides, carotid bruit and unequal BP recording in both upper limbs (right arm 80/70 mmHg and left arm 100/60 mmHg). Rest of the systemic examination was unremarkable. Fundus fluorescein angiography (FFA) revealed multiple 360-degree peripheral capillary nonperfusion areas in both eyes, microaneurysms in the mid-periphery, and leakage from type 3 anastomosis in the left eye (Fig. 2 ). Further investigation via carotid Doppler ultrasonography revealed diffuse circumferential mural thickening of the bilateral common carotid artery (CCA), internal carotid artery (ICA), and external carotid artery (ECA). Macular scan with Spectral domain-optical coherence tomography with enhanced depth imaging (SD-OCT with EDI) both eyes was normal. OCT angiography of right eye showed normal foveal avascular zone in the superficial vascular plexus and deep vascular plexus, however the vascular plexus (superficial as well as deep) of left eye showed a distorted foveal avascular zone with capillary dropout areas which were more prominent temporally (Fig. 3 ). Computed tomography angiography (CTA) revealed diffuse mural thickening of the aorta and near total occlusion of CCA, ICA, ECA, subclavian artery, and left vertebral artery (Fig. 4 ). Laboratory evaluation revealed raised acute phase reactant (C-reactive protein – 15 mg/dL) and negative anti-nuclear antibody. His positron emission tomography scan did not reveal significant fluorodeoxyglucose uptake in aorta and its branches. On the basis of these findings, a diagnosis of TAK with stage 4 TR was made and the patient was managed with intravenous methylprednisolone followed by oral steroids and steroid sparing agents. He also underwent pan retinal photocoagulation in both eyes, along with an intravitreal injection of ranibizumab in the left eye (Fig. 5 ). Discussion and conclusion Ocular involvement in the TAK occurs due to ischemia resulting from narrowing or occlusion of the aorta, carotid, and distal branches. Ocular symptoms can range from transient visual disturbances to more severe conditions, such as TR, which can lead to vision loss. The prevalence of ocular manifestations in the TAK varies widely, ranging from 16–35% of cases [ 5 ]. In some cases, ocular symptoms may be the initial presenting feature [ 6 ], as observed in our patient, who showed progressive blurring of vision as the primary complaint over nine months, accompanied by systemic symptoms such as syncope and limb claudication. This underscores the importance of considering TAK in patients with unexplained ocular symptoms, especially when associated with systemic signs of syncope and limb claudication. The associations of ocular symptoms with upper limb claudication and syncope are significant, as these systemic manifestations often point to underlying vascular involvement, particularly of the aortic arch and its branches. The ischemic changes leading to TR are frequently associated with severe stenosis or occlusion of the carotid or subclavian arteries [ 3 ]. Our patient’s combination of bilateral ocular symptoms, upper limb claudication, and syncope is indicative of severe vascular compromise, likely due to extensive involvement of the aortic arch branches. This correlation between ocular findings and systemic symptoms can be critical for early diagnosis and management. Ocular examination of the TAK typically reveals specific retinal changes, which are classified into four stages of TR, with stage 4 being the most severe, involving complications such as retinal ischemia, neovascularization, and vitreous hemorrhage. Additionally, vascular anastomoses in TRs are categorized into three types on the basis of their origin and location. [6] Our patient presented with stage 4 TR, characterized by multiple microaneurysms in the mid periphery and peripheral capillary nonperfusion areas on fluorescein angiography along with type 3 vascular anastomosis with a wreath-like anastomosis near the optic disc in the left eye. The presence of type 3 anastomoses, indicative of long-standing ischemia, was consistent with the advanced disease stage observed in this case. These findings highlight the critical role of comprehensive ocular examinations in identifying the severity and progression of TAK. Differentiating the TAK from other vasculitides and systemic conditions is essential because of overlapping clinical features. Conditions such as giant cell arteritis, polyarteritis nodosa, and systemic lupus erythematosus can present with similar vascular and ocular symptoms but differ in demographic characteristics, systemic involvement, and laboratory findings [ 7 ]. In our patient, a combination of clinical examination, imaging studies, and laboratory tests ruled out other conditions, confirming the diagnosis of TAK. The treatment primarily involves high-dose corticosteroids to control active inflammation and prevent further vascular damage, with the use of steroid-sparing agents such as methotrexate or azathioprine for long-term management [ 8 – 10 ]. In our patient, prompt initiation of corticosteroids and immunosuppressive agents, along with pan retinal photocoagulation, was crucial in managing ocular complications and systemic disease. In conclusion, TAK, though rare, should be considered in patients presenting with unexplained ocular symptoms, especially when accompanied by systemic signs such as limb claudication and syncopal attacks. A thorough clinical evaluation, supported by advanced imaging techniques, is essential for early diagnosis and treatment. Timely intervention with systemic corticosteroids, immunosuppressive agents and retinal laser photocoagulation can significantly improve outcomes, potentially preventing irreversible visual loss and other serious complications. Abbreviations TAK Takayasu arteritis TR Takayasu retinopathy FFA Fundus fluorescein angiography CCA Common carotid artery ICA Internal carotid artery ECA External carotid artery SD-OCT Spectral domain optical coherence tomography EDI – Enhanced depth imaging OCTA Optical coherence tomography angiography Declarations Acknowledgements None. Authors’ contributions VR, VS drafted the manuscript and collected patient information, KK, ET edited the photos, RG, AM, JK critically revised the manuscript and completed the project. All authors read and approved the final manuscript. Funding Nil Data availability No datasets were generated or analysed during the current study. Ethics approval and consent to participate This study adhered to the tenets of the Declaration of Helsinki. The institutional review board of Command hospital eastern command, Kolkata, India did not require an ethics committee review process to report this case. Consent for publication Written informed consent was obtained from the patient. Competing interests The authors declare no competing interests References Pallangyo P, Epafra E, Nicholaus P, Lyimo F, Kazahura P, Janabi M. Bilateral ocular ischemia-induced blindness as a presenting manifestation of Takayasu arteritis: a case report. J Med Case Rep. 2017;11(1):153. Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013;17(5):603-606. Kang Z, Xu Z, Wu X, Nie C, Yin J, Mei B. Ischemic stroke as the first manifestation of Takayasu arteritis: high resolution magnetic resonance imaging. Neurol Sci. 2021 Nov;42(11):4821-4824. Ishihara T, Haraguchi G, Tezuka D, Kamiishi T, Inagaki H, Isobe M. Diagnosis and assessment of Takayasu arteritis by multiple biomarkers. Circ J. 2013;77:477–83. Ambiya V, Kumar A, Sharma VK, Sharma A. Bilateral Takayasu’s retinopathy as the initial presentation of Takayasu’s arteritis. BMJ Case Rep 2024;17:e258688. Shailaja S, Vivek G, Shetty R, Kamath Y. ‘Eye is a window to the pulse’: Bilateral ocular ischaemic syndrome as a presenting manifestation of Takayasu arteritis. BMJ Case Rep. 2013;2013: bcr2013009461. Gonzalez-Gay MA, Barros S, Lopez-Diaz MJ, Garcia-Porrua C, Sanchez-Andrade A, Llorca J. Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients. Medicine (Baltimore). 2005;84(5):269-276. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al; European Vasculitis Study Group. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2009;68(3):318-23. Unizony S, Stone JH, Stone JR. New treatment strategies in large-vessel vasculitis. Curr Opin Rheumatol. 2013;25(1):3-9. Sharma U, Rishi E, Rishi P, Gupta V, Raman R. Posterior segment manifestations of Takayasu arteritis: A narrative review. Indian J Ophthalmol. 2024;72(5):637-647. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 24 Oct, 2025 Read the published version in BMC Ophthalmology → Version 1 posted Editorial decision: Revision requested 15 Sep, 2025 Reviews received at journal 30 Aug, 2025 Reviewers agreed at journal 30 Aug, 2025 Reviews received at journal 29 Aug, 2025 Reviewers agreed at journal 29 Aug, 2025 Reviewers agreed at journal 26 Aug, 2025 Reviewers invited by journal 26 Aug, 2025 Editor assigned by journal 25 Aug, 2025 Submission checks completed at journal 25 Aug, 2025 First submitted to journal 22 Aug, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-7438009","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":508086724,"identity":"159f0233-db1a-4bb3-81c2-99fdada4831b","order_by":0,"name":"Vipin Rana","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA4UlEQVRIiWNgGAWjYLCChAobOX5m5gMMjA3EanlwJs1Ysr0tgXgtjA/bDiduOHPGgDgt/LOBKhPb0hg33Mj5JvFzh40cA/vhoxvwaZE4l2PAkHDOhlnyRu42yV6gCxl40tJu4LXmDFsCQ0JZGhsfUIsEL9CFDRI8Zni1yIO1sB3mYbiR80zyLzFaDM4Awzah7bCEwJkzbNJE2WII1nImzQAYyMbWsm1pxmyE/CJ3hrGB8UeFTX0/M/PDm2/bgHHKfvgYfu8zMLD/gDJYJEAkGwHlKID5AymqR8EoGAWjYOQAAHhPTXB4dBG2AAAAAElFTkSuQmCC","orcid":"","institution":"Command Hospital, (Eastern Command)","correspondingAuthor":true,"prefix":"","firstName":"Vipin","middleName":"","lastName":"Rana","suffix":""},{"id":508086725,"identity":"e80d68b2-6488-486f-b67d-f1a9e11c3af8","order_by":1,"name":"Vikas Sharma","email":"","orcid":"","institution":"AFCME","correspondingAuthor":false,"prefix":"","firstName":"Vikas","middleName":"","lastName":"Sharma","suffix":""},{"id":508086726,"identity":"f1888feb-1b24-4b56-9fb9-603bdc5a369b","order_by":2,"name":"Kunal Kishore","email":"","orcid":"","institution":"Command Hospital, (Eastern Command)","correspondingAuthor":false,"prefix":"","firstName":"Kunal","middleName":"","lastName":"Kishore","suffix":""},{"id":508086727,"identity":"c4190bea-9b28-4bce-aeb6-5bcb71cb92b0","order_by":3,"name":"Eram Thakur","email":"","orcid":"","institution":"MH Tejpur","correspondingAuthor":false,"prefix":"","firstName":"Eram","middleName":"","lastName":"Thakur","suffix":""},{"id":508086728,"identity":"e7aa3f6d-0aa0-47b6-a2ad-e1e0a08d6123","order_by":4,"name":"Ranjit Goenka","email":"","orcid":"","institution":"Command Hospital, (Eastern Command)","correspondingAuthor":false,"prefix":"","firstName":"Ranjit","middleName":"","lastName":"Goenka","suffix":""},{"id":508086729,"identity":"d7e45146-2aed-4cbc-bd70-e631f99cc6d2","order_by":5,"name":"Ashish Markan","email":"","orcid":"","institution":"All India Institute of Medical Education and Research","correspondingAuthor":false,"prefix":"","firstName":"Ashish","middleName":"","lastName":"Markan","suffix":""},{"id":508086730,"identity":"2d49bb2b-14dd-45b3-ad0f-8703b8ac1c1f","order_by":6,"name":"Jaya Kuashik","email":"","orcid":"","institution":"MH Panagarh, (Eastern Command)","correspondingAuthor":false,"prefix":"","firstName":"Jaya","middleName":"","lastName":"Kuashik","suffix":""}],"badges":[],"createdAt":"2025-08-23 02:23:07","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7438009/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7438009/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12886-025-04446-5","type":"published","date":"2025-10-24T16:17:28+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":90473733,"identity":"ed478b00-8d43-47d2-9f50-8733b48b8f69","added_by":"auto","created_at":"2025-09-03 06:39:33","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":311502,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eA) \u003c/strong\u003eUltra-wide field color fundus photograph of the right eye displaying multiple mid-peripheral microaneurysms, which are more clearly visible in the inset. \u003cstrong\u003eB) \u003c/strong\u003eUltra-wide field color fundus photograph of the left eye, demonstrating multiple mid-peripheral microaneurysms along with a wreath-like arteriovenous formation at the optic disc, which is also more clearly visible in the inset and indicated by blue arrows.\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-7438009/v1/57458eddf0aa79fa23a74e22.png"},{"id":90473719,"identity":"73590271-bcce-49f7-b841-479d6f71477f","added_by":"auto","created_at":"2025-09-03 06:39:29","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":376502,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eUltra-wide field fundus fluorescein angiogram (FFA) of the right eye: A)\u003c/strong\u003eEarly venous phase showing increased arteriovenous filling, with retinal vessels filled only up to the midperiphery (leading edge sign). A hyperfluorescent disc and a hyperfluorescent area in the superonasal quadrant are noted, along with staining of microaneurysms in the midperiphery and capillary non-perfusion areas in all quadrants beyond the midperiphery. \u003cstrong\u003eB)\u003c/strong\u003eLate phase revealing optic disc hyperfluorescence, increased leakage in the superonasal region, staining of midperipheral microaneurysms, and persistence of non-perfused capillary areas.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eUltra-wide field fundus fluorescein angiogram (FFA) of the left eye: \u003c/strong\u003e\u0026nbsp;\u003cstrong\u003eC)\u003c/strong\u003e Showing delayed choroidal filling and delayed arterial filling (leading edge sign). \u003cstrong\u003eD)\u003c/strong\u003e Early venous phase demonstrating disc staining, leakage from a wreath-like arteriovenous anastomosis, staining of microaneurysms in the midperiphery, and capillary non-perfusion areas in all quadrants beyond the midperiphery.\u003c/p\u003e","description":"","filename":"floatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-7438009/v1/20366ea36fb3f32ed789a917.png"},{"id":90473714,"identity":"528f90b8-83e4-49e7-b7c7-cb074e6e324a","added_by":"auto","created_at":"2025-09-03 06:39:29","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":509265,"visible":true,"origin":"","legend":"\u003cp\u003eSpectral domain-optical coherence tomography with enhanced depth imaging (SD-OCT with EDI) of the right eye macula shows: \u003cstrong\u003eA) \u003c/strong\u003eNormal architecture with a normal foveal contour. \u003cstrong\u003eB)\u003c/strong\u003e OCT angiography showing a normal foveal avascular zone in the superficial vascular plexus. \u003cstrong\u003eC) \u003c/strong\u003eand deep vascular plexus. \u003cstrong\u003eD)\u003c/strong\u003e SD-OCT with EDI of the left eye showing normal architecture with a normal foveal contour. However, \u003cstrong\u003eE)\u003c/strong\u003e OCT angiography of the superficial vascular plexus shows a distorted foveal avascular zone with capillary dropout areas, more prominent temporally, \u003cstrong\u003eF) \u003c/strong\u003esimilar findings are seen in deep vascular plexus.\u003c/p\u003e","description":"","filename":"floatimage3.png","url":"https://assets-eu.researchsquare.com/files/rs-7438009/v1/621e2dc0c414f175885e2b5a.png"},{"id":90473729,"identity":"8aedde41-1413-4eb8-ae95-c2db92c5f728","added_by":"auto","created_at":"2025-09-03 06:39:31","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":165893,"visible":true,"origin":"","legend":"\u003cp\u003eComputed tomography angiography demonstrating diffuse mural thickening of the right and left common carotid arteries (approximately 90%) (indicated by white arrows).\u003c/p\u003e","description":"","filename":"floatimage4.png","url":"https://assets-eu.researchsquare.com/files/rs-7438009/v1/26caffcf3358e9d84acaf883.png"},{"id":90473713,"identity":"39bc800d-8a49-4dbc-ac28-a8031410a873","added_by":"auto","created_at":"2025-09-03 06:39:29","extension":"png","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":422602,"visible":true,"origin":"","legend":"\u003cp\u003eUltra-wide field color fundus photographs of the right eye (A) and left eye (B), showing laser photocoagulation scars in the equatorial, midperipheral, and peripheral retina, along with regressed neovascularization and arteriovenous shunt vessels.\u003c/p\u003e","description":"","filename":"floatimage5.png","url":"https://assets-eu.researchsquare.com/files/rs-7438009/v1/b013eb03f2aa3e4cb2bd8260.png"},{"id":94490685,"identity":"24b7d31e-d134-4cd8-aaf1-395bca54c750","added_by":"auto","created_at":"2025-10-27 17:13:42","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2243332,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7438009/v1/436ca733-dd6d-4069-9df0-574d0ba1cfc9.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"When the Eyes Pave the Way: Takayasu Retinopathy as the First Sign of Takayasu Arteritis","fulltext":[{"header":"Background","content":"\u003cp\u003eTakayasu arteritis (TAK) is an inflammatory disease of unknown etiology characterized by granulomatous vasculitis affecting the aorta, its main branches and the arteries. It occurs commonly in women of childbearing age [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. The disease progresses through inflammatory stages that can lead to segmental stenosis, occlusion, dilatation, or aneurysm, resulting in a variety of systemic symptoms, such as extremity pain, claudication, bruits, reduced or absent pulses, and alterations in blood pressure. While TAK typically progresses slowly, it can sometimes present acutely with severe complications such as sudden visual loss or stroke [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Ocular manifestations of TAK, which are often due to reduced blood flow from carotid artery occlusion, include transient visual disturbances, anterior segment ischemia, Takayasu retinopathy (TR), and ischemic optic neuropathy. Additionally, renal artery involvement can lead to hypertensive retinopathy [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eThis report presents a rare case of TA in which decreased vision was the initial and predominant symptom. This ocular manifestation was accompanied by syncope, unequal blood pressures in both upper limbs and bilateral upper limb claudication. Thus, this case underscores the importance of recognizing ocular symptoms as potential early indicators of this systemic vasculitis, particularly when accompanied by signs of vascular compromise.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 29-year-old male presented to our tertiary care hospital with complaints of progressive blurring of vision in both eyes, which was more pronounced in the left eye, over the past nine months. He also reported episodes of presyncope lasting 8–10 seconds without any loss of consciousness or focal neurological deficits for five months. Additionally, he experienced unilateral pain in the left upper limb, extending from the shoulder to the hand. There was no history of constitutional symptoms such as weakness, fever, or weight loss.\u003c/p\u003e\u003cp\u003eOn examination, his best-corrected visual acuity was 20/20 in the right eye and 20/50 in the left eye. The patient’s intraocular pressure was 12 mmHg in both eyes. The anterior segment examination of both eyes was unremarkable. Fundus examination revealed multiple microaneurysms in the mid-peripheral retina across all quadrants, along with a wreath-like arteriovenous anastomosis near the left optic disc (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eSystemic examination revealed absent brachial and radial pulses on both sides, carotid bruit and unequal BP recording in both upper limbs (right arm 80/70 mmHg and left arm 100/60 mmHg). Rest of the systemic examination was unremarkable.\u003c/p\u003e\u003cp\u003eFundus fluorescein angiography (FFA) revealed multiple 360-degree peripheral capillary nonperfusion areas in both eyes, microaneurysms in the mid-periphery, and leakage from type 3 anastomosis in the left eye (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e\u003cp\u003eFurther investigation via carotid Doppler ultrasonography revealed diffuse circumferential mural thickening of the bilateral common carotid artery (CCA), internal carotid artery (ICA), and external carotid artery (ECA). Macular scan with Spectral domain-optical coherence tomography with enhanced depth imaging (SD-OCT with EDI) both eyes was normal. OCT angiography of right eye showed normal foveal avascular zone in the superficial vascular plexus and deep vascular plexus, however the vascular plexus (superficial as well as deep) of left eye showed a distorted foveal avascular zone with capillary dropout areas which were more prominent temporally (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eComputed tomography angiography (CTA) revealed diffuse mural thickening of the aorta and near total occlusion of CCA, ICA, ECA, subclavian artery, and left vertebral artery (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e\u003cp\u003eLaboratory evaluation revealed raised acute phase reactant (C-reactive protein – 15 mg/dL) and negative anti-nuclear antibody. His positron emission tomography scan did not reveal significant fluorodeoxyglucose uptake in aorta and its branches.\u003c/p\u003e\u003cp\u003eOn the basis of these findings, a diagnosis of TAK with stage 4 TR was made and the patient was managed with intravenous methylprednisolone followed by oral steroids and steroid sparing agents. He also underwent pan retinal photocoagulation in both eyes, along with an intravitreal injection of ranibizumab in the left eye (Fig.\u0026nbsp;\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e5\u003c/span\u003e).\u003c/p\u003e\u003cp\u003e\u003c/p\u003e"},{"header":"Discussion and conclusion","content":"\u003cp\u003eOcular involvement in the TAK occurs due to ischemia resulting from narrowing or occlusion of the aorta, carotid, and distal branches. Ocular symptoms can range from transient visual disturbances to more severe conditions, such as TR, which can lead to vision loss. The prevalence of ocular manifestations in the TAK varies widely, ranging from 16–35% of cases [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. In some cases, ocular symptoms may be the initial presenting feature [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e], as observed in our patient, who showed progressive blurring of vision as the primary complaint over nine months, accompanied by systemic symptoms such as syncope and limb claudication. This underscores the importance of considering TAK in patients with unexplained ocular symptoms, especially when associated with systemic signs of syncope and limb claudication.\u003c/p\u003e\u003cp\u003eThe associations of ocular symptoms with upper limb claudication and syncope are significant, as these systemic manifestations often point to underlying vascular involvement, particularly of the aortic arch and its branches. The ischemic changes leading to TR are frequently associated with severe stenosis or occlusion of the carotid or subclavian arteries [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. Our patient’s combination of bilateral ocular symptoms, upper limb claudication, and syncope is indicative of severe vascular compromise, likely due to extensive involvement of the aortic arch branches. This correlation between ocular findings and systemic symptoms can be critical for early diagnosis and management.\u003c/p\u003e\u003cp\u003eOcular examination of the TAK typically reveals specific retinal changes, which are classified into four stages of TR, with stage 4 being the most severe, involving complications such as retinal ischemia, neovascularization, and vitreous hemorrhage. Additionally, vascular anastomoses in TRs are categorized into three types on the basis of their origin and location.\u003csup\u003e[6]\u003c/sup\u003e Our patient presented with stage 4 TR, characterized by multiple microaneurysms in the mid periphery and peripheral capillary nonperfusion areas on fluorescein angiography along with type 3 vascular anastomosis with a wreath-like anastomosis near the optic disc in the left eye. The presence of type 3 anastomoses, indicative of long-standing ischemia, was consistent with the advanced disease stage observed in this case. These findings highlight the critical role of comprehensive ocular examinations in identifying the severity and progression of TAK.\u003c/p\u003e\u003cp\u003eDifferentiating the TAK from other vasculitides and systemic conditions is essential because of overlapping clinical features. Conditions such as giant cell arteritis, polyarteritis nodosa, and systemic lupus erythematosus can present with similar vascular and ocular symptoms but differ in demographic characteristics, systemic involvement, and laboratory findings [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. In our patient, a combination of clinical examination, imaging studies, and laboratory tests ruled out other conditions, confirming the diagnosis of TAK. The treatment primarily involves high-dose corticosteroids to control active inflammation and prevent further vascular damage, with the use of steroid-sparing agents such as methotrexate or azathioprine for long-term management [\u003cspan additionalcitationids=\"CR9\" citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e–\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. In our patient, prompt initiation of corticosteroids and immunosuppressive agents, along with pan retinal photocoagulation, was crucial in managing ocular complications and systemic disease.\u003c/p\u003e\u003cp\u003eIn conclusion, TAK, though rare, should be considered in patients presenting with unexplained ocular symptoms, especially when accompanied by systemic signs such as limb claudication and syncopal attacks. A thorough clinical evaluation, supported by advanced imaging techniques, is essential for early diagnosis and treatment. Timely intervention with systemic corticosteroids, immunosuppressive agents and retinal laser photocoagulation can significantly improve outcomes, potentially preventing irreversible visual loss and other serious complications.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003e\u003cstrong\u003eTAK\u003c/strong\u003e Takayasu arteritis\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTR\u003c/strong\u003e\u0026nbsp; \u0026nbsp;Takayasu retinopathy\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFFA\u003c/strong\u003e Fundus fluorescein angiography\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCCA\u003c/strong\u003e Common carotid artery\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eICA\u003c/strong\u003e Internal carotid artery\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eECA\u003c/strong\u003e\u0026nbsp; \u0026nbsp;External carotid artery\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eSD-OCT\u003c/strong\u003e Spectral domain optical coherence tomography\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEDI\u003c/strong\u003e \u0026ndash; Enhanced depth imaging\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eOCTA\u003c/strong\u003e Optical coherence tomography angiography\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNone.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eVR, VS drafted the manuscript and collected patient information, KK, ET edited the photos, RG, AM, JK critically revised the manuscript and completed the project. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNil\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNo datasets were generated or analysed during the current study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study adhered to the tenets of the Declaration of Helsinki. The institutional review board of Command hospital eastern command, Kolkata, India did not require an ethics committee review process to report this case.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no competing interests\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003ePallangyo P, Epafra E, Nicholaus P, Lyimo F, Kazahura P, Janabi M. Bilateral ocular ischemia-induced blindness as a presenting manifestation of Takayasu arteritis: a case report. J Med Case Rep. 2017;11(1):153.\u003c/li\u003e\n\u003cli\u003eJennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013;17(5):603-606.\u003c/li\u003e\n\u003cli\u003eKang Z, Xu Z, Wu X, Nie C, Yin J, Mei B. Ischemic stroke as the first manifestation of Takayasu arteritis: high resolution magnetic resonance imaging. Neurol Sci. 2021 Nov;42(11):4821-4824.\u003c/li\u003e\n\u003cli\u003eIshihara T, Haraguchi G, Tezuka D, Kamiishi T, Inagaki H, Isobe M. Diagnosis and assessment of Takayasu arteritis by multiple biomarkers. Circ J. 2013;77:477\u0026ndash;83.\u003c/li\u003e\n\u003cli\u003eAmbiya V, Kumar A, Sharma VK, Sharma A. Bilateral Takayasu\u0026rsquo;s retinopathy as the initial presentation of Takayasu\u0026rsquo;s arteritis. BMJ Case Rep 2024;17:e258688.\u003c/li\u003e\n\u003cli\u003eShailaja S, Vivek G, Shetty R, Kamath Y. \u0026lsquo;Eye is a window to the pulse\u0026rsquo;: Bilateral ocular ischaemic syndrome as a presenting manifestation of Takayasu arteritis. BMJ Case Rep. 2013;2013: bcr2013009461.\u003c/li\u003e\n\u003cli\u003eGonzalez-Gay MA, Barros S, Lopez-Diaz MJ, Garcia-Porrua C, Sanchez-Andrade A, Llorca J. Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients. Medicine (Baltimore). 2005;84(5):269-276.\u003c/li\u003e\n\u003cli\u003eMukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al; European Vasculitis Study Group. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2009;68(3):318-23.\u003c/li\u003e\n\u003cli\u003eUnizony S, Stone JH, Stone JR. New treatment strategies in large-vessel vasculitis. Curr Opin Rheumatol. 2013;25(1):3-9. \u003c/li\u003e\n\u003cli\u003eSharma U, Rishi E, Rishi P, Gupta V, Raman R. Posterior segment manifestations of Takayasu arteritis: A narrative review. Indian J Ophthalmol. 2024;72(5):637-647.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-ophthalmology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"boph","sideBox":"Learn more about [BMC Ophthalmology](http://bmcophthalmol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/boph","title":"BMC Ophthalmology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Takayasu arteritis, Takayasu retinopathy, Vasculitis, Claudication","lastPublishedDoi":"10.21203/rs.3.rs-7438009/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7438009/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e\u003cp\u003eTakayasu arteritis (TAK) is a granulomatous large-vessel vasculitis of unknown etiology that predominantly affects the aorta and its branches. Ocular involvement occurs due to ischemia from vascular stenosis or occlusion and may present as transient visual loss, Takayasu retinopathy (TR), or ischemic optic neuropathy. In rare cases, ocular symptoms can be the initial manifestation, underscoring the importance of early recognition.\u003c/p\u003e\u003ch2\u003eCase Presentation\u003c/h2\u003e\u003cp\u003eWe report a 29-year-old male who presented with progressive blurring of vision, more marked in the left eye, for nine months. Systemic symptoms included presyncope and upper limb claudication. Ocular examination revealed multiple mid-peripheral microaneurysms and a wreath-like type 3 arteriovenous anastomosis near the left optic disc. Fundus fluorescein angiography showed 360\u0026deg; peripheral capillary nonperfusion, while Optical coherence tomography angiography demonstrated capillary dropout in the macular area in left eye. Carotid Doppler and CT angiography revealed diffuse mural thickening and near-total occlusion of major aortic arch branches. Raised C-reactive protein with negative anti-nuclear antibodies supported the diagnosis. Based on these findings, a diagnosis of TAK with stage 4 TR was made. The patient was treated with intravenous corticosteroids followed by immunosuppressants, along with panretinal photocoagulation and intravitreal ranibizumab in the left eye.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e\u003cp\u003eThis case highlights decreased vision as the initial and predominant manifestation of TAK, accompanied by systemic vascular symptoms. The presence of advanced TR with type 3 anastomosis reflected severe, chronic ischemia. Recognizing ocular signs in conjunction with systemic features such as claudication and syncope is vital for early diagnosis. Prompt systemic immunosuppression with adjunctive ocular interventions can prevent irreversible visual loss and systemic complications.\u003c/p\u003e","manuscriptTitle":"When the Eyes Pave the Way: Takayasu Retinopathy as the First Sign of Takayasu Arteritis","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-09-03 06:38:44","doi":"10.21203/rs.3.rs-7438009/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-09-15T06:54:57+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-08-30T07:57:18+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"142250064525375630087614551983346631196","date":"2025-08-30T06:48:47+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-08-29T15:26:03+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"147286186489355548504950470711492257658","date":"2025-08-29T15:09:37+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"264584387761821691643846304997453859099","date":"2025-08-26T13:03:11+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-08-26T12:46:37+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-08-25T07:00:02+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-08-25T06:58:49+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Ophthalmology","date":"2025-08-23T02:09:42+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-ophthalmology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"boph","sideBox":"Learn more about [BMC Ophthalmology](http://bmcophthalmol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/boph","title":"BMC Ophthalmology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"6adecf34-7d94-4e4e-8aef-3a9582715c24","owner":[],"postedDate":"September 3rd, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-10-27T16:28:53+00:00","versionOfRecord":{"articleIdentity":"rs-7438009","link":"https://doi.org/10.1186/s12886-025-04446-5","journal":{"identity":"bmc-ophthalmology","isVorOnly":false,"title":"BMC Ophthalmology"},"publishedOn":"2025-10-24 16:17:28","publishedOnDateReadable":"October 24th, 2025"},"versionCreatedAt":"2025-09-03 06:38:44","video":"","vorDoi":"10.1186/s12886-025-04446-5","vorDoiUrl":"https://doi.org/10.1186/s12886-025-04446-5","workflowStages":[]},"version":"v1","identity":"rs-7438009","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7438009","identity":"rs-7438009","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}