A Challenging case of Takayasu’s Arteritis in a young male with various manifestations and poor outcome

A Challenging case of Takayasu’s Arteritis in a young male with various manifestations and poor outcome | 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 A Challenging case of Takayasu’s Arteritis in a young male with various manifestations and poor outcome Shabnam Boudagh, Marzieh Mirtajaddini, Simin Almasi, Nahid Rezayean, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4442061/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 12 Nov, 2024 Read the published version in BMC Cardiovascular Disorders → Version 1 posted 12 You are reading this latest preprint version Abstract A 17-year-old boy complaining of progressive dyspnea, fever, palpitations, a 22 mm Hg blood pressure difference between the 2 arms, and arm claudication. He had a history of psoriasis-like skin lesions and bronchiectasis. Echocardiography revealed a reduced left ventricular ejection fraction, severe eccentric aortic insufficiency, circumferential aortic wall thickening, and a dilated ascending aorta with severe atherosclerotic changes. Based on imaging findings, a TA diagnosis was suggested. During his follow-up, the patient developed strabismus, blurred vision, and right sixth cranial nerve paralysis and went into a deep coma. Unfortunately, after 6 months of treatment, he expired due to COVID-19 infection. Case Report Takayasu Cardiovascular disease Vasculitis Figures Figure 1 Figure 2 Figure 3 INTRODUCTION Takayasu’s arteritis (TA) is classified as a rare granulomatous panarteritis affecting the aorta and its major branches. It is predominant in women and has a peak incidence between ages 20 and 40. 1 The most frequent cardiac manifestations of TA are valvular abnormalities, found in more than 60% of patients, 2 and aortic regurgitation is the most common type of valvular heart disease 3 . Other non-common cardiac presentations are acute myocardial infarction, myocarditis, pericarditis, and pulmonary hypertension. 4 Noninvasive imaging techniques, such as magnetic resonance angiography (MRA) and computed tomography angiography (CTA), are playing important role for diagnosing TA and extension of vascular involvement. CASE DESCRIPTION A 17-year-old boy presented to the emergency department complaining of progressive dyspnea with New York Heart Association functional class III. Over the prior decade, he had visited medical centers on numerous occasions. At 6 years of age, he was taken to a pediatrician due to dyspnea, urticaria, and food allergies. Two weeks after treatment initiation for allergies, his symptoms changed to skin erythematous plaques with thick scales that covered his elbows and thighs, suspicious of psoriasis. But he discontinued all treatments without further evaluation of skin lesions. Nevertheless, his dyspnea progressed to the extent that after 2 years, he was unable to participate in football games with his peers. He was taken to a medical center, where he received treatment for bronchiectasis and asthma. On arrival at our facility, at age 17, with progressive dyspnea and right-arm claudication, a 22 mm Hg blood pressure difference between the arms, symmetrical and bounding peripheral pulses, tachycardia (103 bpm), oral temperature of 38 ℃, and oxygen saturation of 98% in room air. He was eupneic at rest and had mild basal crackles on pulmonary auscultation and early diastolic heart murmurs (III/VI) on cardiac auscultation. Electrocardiography demonstrated sinus tachycardia. A chest X-ray showed cardiomegaly and bilateral infiltration. Laboratory evaluations revealed a white blood cell count of 14,200 cells/mm 3 (neutrophil seg = 72%, lymphocyte = 7%, and eosinophil = 15%), a C-reactive protein level of 46 mg/L, an erythrocyte sedimentation rate of 23 and a B-type natriuretic peptide (BNP) level of 4360 pg/mL, with no other alterations. Transthoracic echocardiography revealed severe left ventricular (LV) dilation with severe LV systolic dysfunction (global ejection fraction = 20%) and global hypokinesia; severe eccentric aortic insufficiency; circumferential aortic wall thickening; a dilated ascending aorta (5.2 cm) with severe atherosclerotic changes; holodiastolic flow reversal in the descending aorta; a narrowing just distal to the origin of the subclavian artery with systolic turbulence and without a diastolic tail; circumferential thickening of the abdominal aorta with atherosclerotic changes and moderate pulmonary hypertension (Fig. 1 ). The patient was admitted to the cardiac care unit. A CTA of the total aorta illustrated dilation of the ascending aorta, calcification of the aortic wall from the ascending to the mid-descending thoracic aorta, and irregularity of the aortic wall (Fig. 2 ). MRA also revealed a dilated ascending aorta (max at 54 mm at the right pulmonary artery level), a thickened and irregular aortic wall, atherosclerotic or intruding lesions from the vessel wall into the arch and descending aorta causing narrowing in the narrowest part of the arch (11 mm), and multiple aneurysmal regions in the ascending and descending aorta. No evidence of myocardial inflammation or edema was detected in short tau inversion recovery (STIR)-T2. In the gadolinium study, no thrombus was evident in the LV and right ventricle in the early phase, while the late phase revealed mid-wall enhancement in the mid-septal wall (Fig. 3 ). The patient’s clinical and imaging findings, as well as rheumatology consultations, confirmed the diagnosis of TA. After discharge, 2weaks later, the patient was referred to a rheumatology center. He received Methylprednisolone pulse therapy. Laboratory studies also revealed negative antineutrophil cytoplasmic antibodies (ANCA), rheumatoid factor (RF), antinuclear antibodies (ANA), and HLA-B27, as well as normal C3 and C4. After an acceptable response to corticosteroids, he was discharged to continue treatment at home via monotherapy with oral prednisolone. Two months later, the patient experienced changes in his symptoms; lethargy, ataxia, blurred vision, strabismus, right sixth cranial nerve paralysis, hallucination, and muscle weakness with a decreased muscle force (4/5, symmetrically, both upper and lower limbs) were recorded. Based on ophthalmology evaluation, ischemic ocular TA was mentioned. Moreover, in neurologic evaluation, the brain CT scan showed hypodensity in the left basal ganglia and right parietal. A brain MRI revealed some degrees of vasculitis in the major branches of the cerebral arteries. Methylprednisolone pulse therapy was started, then mycophenolate mofetil (500 mg BID) and infliximab (anti-TNF recombinant antibody) were added to the basic treatment with oral prednisolone. In follow up, the patient experienced one episode of deep coma, only responsive to methylprednisolone pulse therapy. Eventually, 2 months later, Ipsilateral swelling of the face, dyspnea, multiple mouth aphthous ulcers, epistaxis and loss of consciousness occurred. The initial evaluation showed a positive COVID-19 polymerase chain reaction test and mucormycosis with a perforated septum and a saddle nose deformity. Additionally, cytomegalovirus and Klebsiella pneumonia were detected in bronchoscopic specimens. Broad-spectrum antibiotics and ganciclovir, dexamethasone, and remdesivir comprised the principal treatments. However, after 14 days, the patient died in the setting of bradycardia and asystole. DISCUSSION In general, we encountered a young boy with Takayasu's arteritis, presenting with heart failure, severe aortic regurgitation, aortic-cerebral-ocular vasculitis without coronary involvement, and multiple flare-ups. Unfortunately, he had a dramatic end, succumbing to death due to COVID-19. Based on the American College of Rheumatology (ACR)/the European League against Rheumatism (EULAR) Classification Criteria for TA (2022), the most common features include claudication, angina, vascular bruits, and reduced pulses in the upper extremities, carotid artery abnormalities, and systolic blood pressure differences between arms. 5 Our patient exhibited imaging criteria, claudication, and systolic blood pressure differences, leading to the proposal of Takayasu arteritis as the primary diagnosis. While TA is much more frequent in women aged between 20 and 40, our patient was a young man who became symptomatic at age 17. Unfortunately, the patient never had a meticulous examination of his skin lesions; still, case reports indicate that the concomitance of TA and psoriasis in a patient is, albeit rare, probably not coincidental. Indubitably, more research is needed to elucidate the possible connection between these 2 diseases. TA have also some atypical features. Bronchiectasis is a case in point. The reason for this coexistence has yet to be determined. Other tragic events were cerebral and ocular vasculitis, which manifested as coma and strabismus and highlight the importance of early diagnosis and effective immunosuppressive treatment. Moreover, there are no suitable surgical or endovascular procedures for patients with TA and severe aortic insufficiency, severe calcification of the aorta (defined as a porcelain aorta). Indeed, manipulate friable and inflamed tissue increase the risk of valve detachment and anastomotic aneurysms, as well as manipulation of a porcelain aorta, which increases the risk of stroke and embolization. Despite treatment with corticosteroids, infliximab, and mycophenolate mofetil, our patient’s rheumatologic symptoms continued to worsen. Fortunately, recent years have witnessed the emergence of new targeted biological agents for TA treatment. 6 Nonetheless, the increased infection risk precluded us from utilizing them. The last, but by no means the least, tragic event was our patient’s contracting COVID-19. Immunosuppressive agents, as the integral component of TA control, can increase susceptibility to infections. We commenced the latest universal treatment for COVID-19 promptly. Our patient, however, failed to respond to initial treatments, which indicates the lifesaving role of vaccination in patients with TA. CONCLUSIONS Here, we reported a novel case involving the coexistence of TA, severe aortic insufficiency, porcelain aorta, heart failure, cerebral and ocular vasculitis, and bronchiectasis with challenging management. Further investigations to determine the reasons for these associations will help devise new generations of treatments. In fact, our patient demonstrated the importance of early diagnosis, effective immunosuppressive treatment, new endovascular or surgical strategies and close follow-up. Declarations Consent for publication : Informed consent obtained from patient's parents. Undoubtedly, we would provide the copy, if specifically requested by the journal. Declarations of interest: None Declaration of Generative AI and AI-assisted technologies in the writing process: None Ethics approval and consent to participate: Not applicable Consent for publication: Not applicable Availability of data and materials: Not applicable Competing interests: Not applicable Funding: Not applicable Authors' contributions: Not applicable Acknowledgements: Not applicable Availability of data and materials: All data generated or analyzed during this study are included in this published article [and its supplementary information files]. Clinical trial number: This article is just a case description without clinical trial. (Number: 0) References Florence A. Aeschlimann, Rae S. M. Yeung and Ronald M. Laxer, An Update on Childhood-Onset Takayasu Arteritis, 2022 Apr 13. doi: 10.3389/fped.2022.872313 Ren Y, Du J, Guo X, Liu O, Liu W, Qi G, et al. Cardiac valvular involvement of Takayasu arteritis. Clin Rheumatol. (2021) 40:653–60. doi: 10.1007/s10067-020-05290-2 Lee GY, Jang SY, Ko SM, Kim EK, Lee SH, Han H, et al. Cardiovascular manifestations of Takayasu arteritis and their relationship to the disease activity: analysis of 204 Korean patients at a single center. Int J Cardiol. (2012) 159:14–20. doi: 10.1016/j.ijcard.2011.01.094 Francesca Regola, Martina Uzzo, Paola Toniati, Barbara Trezzi, Renato Alberto Sinico and Franco Franceschini1, Novel Therapies in Takayasu Arteritis, Front. Med., 12 January 2022, Sec. Rheumatology, Volume 8 – 2021, https://doi.org/10.3389/fmed.2021.814075 Peter C Grayson, Cristina Ponte, Ravi Suppiah, Joanna C Robson, Katherine Bates Gibbons, Andrew Judge, Anthea Craven, Sara Khalid, Andrew Hutchings, Debashish Danda, Raashid A Luqmani, Richard A Watts, Peter A Merkel, 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis, BMJ journals, Ann Rheum Dis 2022;81:1654–1660. doi:10.1136/ard-2022-223482 Francesca Regola, Martina Uzzo, Paola Toniati, Barbara Trezzi, Renato Alberto Sinico and Franco Franceschini1, Novel Therapies in Takayasu Arteritis, Front. Med., 12 January 2022, Sec. Rheumatology, Volume 8 – 2021, https://doi.org/10.3389/fmed.2021.814075 Additional Declarations No competing interests reported. Supplementary Files GraphicalAbstract.png Cite Share Download PDF Status: Published Journal Publication published 12 Nov, 2024 Read the published version in BMC Cardiovascular Disorders → Version 1 posted Editorial decision: Revision requested 19 Sep, 2024 Reviews received at journal 15 Sep, 2024 Reviewers agreed at journal 13 Sep, 2024 Reviewers agreed at journal 08 Sep, 2024 Reviews received at journal 04 Aug, 2024 Reviewers agreed at journal 14 Jul, 2024 Reviewers agreed at journal 08 Jul, 2024 Reviewers invited by journal 20 Jun, 2024 Editor invited by journal 18 Jun, 2024 Editor assigned by journal 18 Jun, 2024 Submission checks completed at journal 18 Jun, 2024 First submitted to journal 18 May, 2024 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. 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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-4442061","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":318606824,"identity":"d7de1d2a-cc89-4b50-8f40-7b5a41cf00f5","order_by":0,"name":"Shabnam Boudagh","email":"","orcid":"","institution":"Shaheed Rajaei Cardiovascular Medical and Research Center","correspondingAuthor":false,"prefix":"","firstName":"Shabnam","middleName":"","lastName":"Boudagh","suffix":""},{"id":318606825,"identity":"c5aeb3b4-5c51-4b58-8949-6c1df705a0dd","order_by":1,"name":"Marzieh Mirtajaddini","email":"","orcid":"","institution":"Shaheed Rajaei Cardiovascular Medical and Research Center","correspondingAuthor":false,"prefix":"","firstName":"Marzieh","middleName":"","lastName":"Mirtajaddini","suffix":""},{"id":318606826,"identity":"6561d662-35c3-4f3e-9dde-bb71b4fb5d16","order_by":2,"name":"Simin Almasi","email":"","orcid":"","institution":"Shaheed Rajaei Cardiovascular Medical and Research Center","correspondingAuthor":false,"prefix":"","firstName":"Simin","middleName":"","lastName":"Almasi","suffix":""},{"id":318606828,"identity":"c0e76872-a0b6-4244-bb14-abb71a8e4a28","order_by":3,"name":"Nahid Rezayean","email":"","orcid":"","institution":"Shaheed Rajaei Cardiovascular Medical and Research Center","correspondingAuthor":false,"prefix":"","firstName":"Nahid","middleName":"","lastName":"Rezayean","suffix":""},{"id":318606829,"identity":"d84b6813-fcbd-4536-bc4a-7c2f637fedeb","order_by":4,"name":"Saba Simiyari","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA7ElEQVRIiWNgGAWjYFACHhAhwcAGoj4wMCSQpoVxBglaIICZhxgt8u1nD35g+GMRzcd+9pm0bZtdHj97A+OHjzm4tRicyUuWYGyTyG3jSTeTzm1LLpbsOcAsOXMbHi0MOQYSjA1ALQxpbEAtzIkbbiSwMfPi0SLf/8b4B8MfoBb+Z2zSlm31hLUw3MgxAwYXUIsE0BbGtsOEtRjceJdmkQjyi8QzZsuec8cTZ/YcbMbrF/n+3MM3Pvypy53fn8Z440dZdWI/e/PBDx/xOQwEEiAUiwQjOA0wNhBQjwDMwAgiWvEoGAWjYBSMIAAArPFNaYBSGWoAAAAASUVORK5CYII=","orcid":"","institution":"Shaheed Rajaei Cardiovascular Medical and Research Center","correspondingAuthor":true,"prefix":"","firstName":"Saba","middleName":"","lastName":"Simiyari","suffix":""}],"badges":[],"createdAt":"2024-05-18 18:08:14","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4442061/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4442061/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12872-024-04311-1","type":"published","date":"2024-11-12T15:57:11+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":60355294,"identity":"5181399f-7a65-4b3c-813a-c9ba469698b8","added_by":"auto","created_at":"2024-07-16 00:01:57","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":470406,"visible":true,"origin":"","legend":"\u003cp\u003eTransthoracic echocardiogram obtained at admission. (A) Parasternal long axis view showing dilation of ascending aorta (5.2 cm). (B) Parasternal long axis view showing Al vena contracta: 0.62cm in favor of severe Al. (C) Four chamber view showing LV end diastolic volume index: 114.9 ml/m2. (D) Five chamber view showing Al Doppler. (E) Diastolic flow reversal in descending thoracic aorta by pulse-wave Doppler and end-diastolic velocity is greater than 20 cm/s. (F) A narrowing is seen just distal to the origin of the subclavian artery. (G) The image shows circumferential thickening of the abdominal aorta with atherosclerotic changes and the normal size of the abdominal aorta in the proximal part (2.24 cm). (H) A narrowing is seen just distal to the origin of the subclavian artery with systolic turbulence (the peak pressure gradient = 20 mm Hg) and without a diastolic tail.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-4442061/v1/55aa14510e0a30a6dd9ed4bf.png"},{"id":60354705,"identity":"90be658b-78c3-4f75-9ca8-dc0d20e2a99a","added_by":"auto","created_at":"2024-07-15 23:53:57","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":202311,"visible":true,"origin":"","legend":"\u003cp\u003eThe aortic computed tomography angiography with 2 different volume-rendering technique (VRT) reconstructions reveals dilation of the ascending aorta (the asterisk), calcification of the aortic wall from the ascending to the mid-descending thoracic aorta (the yellow arrow), and irregularity of the aortic wall.\u003c/p\u003e\n\u003cp\u003eAbbreviations: VRT: (Volume Rendering Technique)\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-4442061/v1/6852775ed772108a28591810.png"},{"id":60354706,"identity":"32d49c84-1885-4380-933d-b9ad240610a7","added_by":"auto","created_at":"2024-07-15 23:53:57","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":375241,"visible":true,"origin":"","legend":"\u003cp\u003eTakayasu arteritis is displayed in different cardiac magnetic resonance sequences. (A) The aortic double-oblique candy-cane view demonstrates irregularity of the aortic wall (the yellow arrow) and dilation of the ascending aorta (the asterisk). (B) The short tau inversion recovery (STIR) image shows edema of the aortic wall (the yellow arrow). (C) The magnetic resonance angiography depicts irregularity, localized dilation, and mild narrowing of the aortic arch branches (the white arrows) and irregularity of the aortic wall (the yellow arrow). (D) The late gadolinium enhancement (LGE) sequence illustrates aortic wall fibrosis (the yellow arrow).\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-4442061/v1/8035a9d57bfbc0ebca0b963c.png"},{"id":69274936,"identity":"19b5f1b3-1963-46f7-9352-c7a1661e6afe","added_by":"auto","created_at":"2024-11-18 16:39:54","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1386383,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4442061/v1/2544fe56-af36-4f8f-ab52-682c0947acb3.pdf"},{"id":60354707,"identity":"08e696e6-5473-42da-9309-b265e3cf01cf","added_by":"auto","created_at":"2024-07-15 23:53:57","extension":"png","order_by":1,"title":"","display":"","copyAsset":false,"role":"supplement","size":616111,"visible":true,"origin":"","legend":"","description":"","filename":"GraphicalAbstract.png","url":"https://assets-eu.researchsquare.com/files/rs-4442061/v1/6753e6eaf34f119ca61ec2a9.png"}],"financialInterests":"No competing interests reported.","formattedTitle":"A Challenging case of Takayasu’s Arteritis in a young male with various manifestations and poor outcome","fulltext":[{"header":"INTRODUCTION","content":"\u003cp\u003eTakayasu\u0026rsquo;s arteritis (TA) is classified as a rare granulomatous panarteritis affecting the aorta and its major branches. It is predominant in women and has a peak incidence between ages 20 and 40.\u003csup\u003e\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eThe most frequent cardiac manifestations of TA are valvular abnormalities, found in more than 60% of patients, \u003csup\u003e\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u003c/sup\u003e and aortic regurgitation is the most common type of valvular heart disease\u003csup\u003e\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u003c/sup\u003e. Other non-common cardiac presentations are acute myocardial infarction, myocarditis, pericarditis, and pulmonary hypertension. \u003csup\u003e\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e\u003c/sup\u003e\u003c/p\u003e \u003cp\u003eNoninvasive imaging techniques, such as magnetic resonance angiography (MRA) and computed tomography angiography (CTA), are playing important role for diagnosing TA and extension of vascular involvement.\u003c/p\u003e"},{"header":"CASE DESCRIPTION","content":"\u003cp\u003eA 17-year-old boy presented to the emergency department complaining of progressive dyspnea with New York Heart Association functional class III. Over the prior decade, he had visited medical centers on numerous occasions. At 6 years of age, he was taken to a pediatrician due to dyspnea, urticaria, and food allergies. Two weeks after treatment initiation for allergies, his symptoms changed to skin erythematous plaques with thick scales that covered his elbows and thighs, suspicious of psoriasis. But he discontinued all treatments without further evaluation of skin lesions. Nevertheless, his dyspnea progressed to the extent that after 2 years, he was unable to participate in football games with his peers. He was taken to a medical center, where he received treatment for bronchiectasis and asthma.\u003c/p\u003e \u003cp\u003eOn arrival at our facility, at age 17, with progressive dyspnea and right-arm claudication, a 22 mm Hg blood pressure difference between the arms, symmetrical and bounding peripheral pulses, tachycardia (103 bpm), oral temperature of 38 ℃, and oxygen saturation of 98% in room air. He was eupneic at rest and had mild basal crackles on pulmonary auscultation and early diastolic heart murmurs (III/VI) on cardiac auscultation.\u003c/p\u003e \u003cp\u003eElectrocardiography demonstrated sinus tachycardia. A chest X-ray showed cardiomegaly and bilateral infiltration. Laboratory evaluations revealed a white blood cell count of 14,200 cells/mm\u003csup\u003e3\u003c/sup\u003e (neutrophil seg\u0026thinsp;=\u0026thinsp;72%, lymphocyte\u0026thinsp;=\u0026thinsp;7%, and eosinophil\u0026thinsp;=\u0026thinsp;15%), a C-reactive protein level of 46 mg/L, an erythrocyte sedimentation rate of 23 and a B-type natriuretic peptide (BNP) level of 4360 pg/mL, with no other alterations.\u003c/p\u003e \u003cp\u003eTransthoracic echocardiography revealed severe left ventricular (LV) dilation with severe LV systolic dysfunction (global ejection fraction\u0026thinsp;=\u0026thinsp;20%) and global hypokinesia; severe eccentric aortic insufficiency; circumferential aortic wall thickening; a dilated ascending aorta (5.2 cm) with severe atherosclerotic changes; holodiastolic flow reversal in the descending aorta; a narrowing just distal to the origin of the subclavian artery with systolic turbulence and without a diastolic tail; circumferential thickening of the abdominal aorta with atherosclerotic changes and moderate pulmonary hypertension (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eThe patient was admitted to the cardiac care unit. A CTA of the total aorta illustrated dilation of the ascending aorta, calcification of the aortic wall from the ascending to the mid-descending thoracic aorta, and irregularity of the aortic wall (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eMRA also revealed a dilated ascending aorta (max at 54 mm at the right pulmonary artery level), a thickened and irregular aortic wall, atherosclerotic or intruding lesions from the vessel wall into the arch and descending aorta causing narrowing in the narrowest part of the arch (11 mm), and multiple aneurysmal regions in the ascending and descending aorta. No evidence of myocardial inflammation or edema was detected in short tau inversion recovery (STIR)-T2. In the gadolinium study, no thrombus was evident in the LV and right ventricle in the early phase, while the late phase revealed mid-wall enhancement in the mid-septal wall (Fig.\u0026nbsp;\u003cspan refid=\"Fig6\" class=\"InternalRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eThe patient\u0026rsquo;s clinical and imaging findings, as well as rheumatology consultations, confirmed the diagnosis of TA.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eAfter discharge, 2weaks later, the patient was referred to a rheumatology center. He received Methylprednisolone pulse therapy. Laboratory studies also revealed negative antineutrophil cytoplasmic antibodies (ANCA), rheumatoid factor (RF), antinuclear antibodies (ANA), and HLA-B27, as well as normal C3 and C4. After an acceptable response to corticosteroids, he was discharged to continue treatment at home via monotherapy with oral prednisolone.\u003c/p\u003e \u003cp\u003eTwo months later, the patient experienced changes in his symptoms; lethargy, ataxia, blurred vision, strabismus, right sixth cranial nerve paralysis, hallucination, and muscle weakness with a decreased muscle force (4/5, symmetrically, both upper and lower limbs) were recorded. Based on ophthalmology evaluation, ischemic ocular TA was mentioned. Moreover, in neurologic evaluation, the brain CT scan showed hypodensity in the left basal ganglia and right parietal. A brain MRI revealed some degrees of vasculitis in the major branches of the cerebral arteries. Methylprednisolone pulse therapy was started, then mycophenolate mofetil (500 mg BID) and infliximab (anti-TNF recombinant antibody) were added to the basic treatment with oral prednisolone. In follow up, the patient experienced one episode of deep coma, only responsive to methylprednisolone pulse therapy.\u003c/p\u003e \u003cp\u003eEventually, 2 months later, Ipsilateral swelling of the face, dyspnea, multiple mouth aphthous ulcers, epistaxis and loss of consciousness occurred. The initial evaluation showed a positive COVID-19 polymerase chain reaction test and mucormycosis with a perforated septum and a saddle nose deformity. Additionally, cytomegalovirus and Klebsiella pneumonia were detected in bronchoscopic specimens. Broad-spectrum antibiotics and ganciclovir, dexamethasone, and remdesivir comprised the principal treatments. However, after 14 days, the patient died in the setting of bradycardia and asystole.\u003c/p\u003e"},{"header":"DISCUSSION","content":"\u003cp\u003eIn general, we encountered a young boy with Takayasu's arteritis, presenting with heart failure, severe aortic regurgitation, aortic-cerebral-ocular vasculitis without coronary involvement, and multiple flare-ups. Unfortunately, he had a dramatic end, succumbing to death due to COVID-19.\u003c/p\u003e \u003cp\u003eBased on the American College of Rheumatology (ACR)/the European League against Rheumatism (EULAR) Classification Criteria for TA (2022), the most common features include claudication, angina, vascular bruits, and reduced pulses in the upper extremities, carotid artery abnormalities, and systolic blood pressure differences between arms. \u003csup\u003e\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e\u003c/sup\u003e Our patient exhibited imaging criteria, claudication, and systolic blood pressure differences, leading to the proposal of Takayasu arteritis as the primary diagnosis.\u003c/p\u003e \u003cp\u003eWhile TA is much more frequent in women aged between 20 and 40, our patient was a young man who became symptomatic at age 17.\u003c/p\u003e \u003cp\u003eUnfortunately, the patient never had a meticulous examination of his skin lesions; still, case reports indicate that the concomitance of TA and psoriasis in a patient is, albeit rare, probably not coincidental. Indubitably, more research is needed to elucidate the possible connection between these 2 diseases.\u003c/p\u003e \u003cp\u003eTA have also some atypical features. Bronchiectasis is a case in point. The reason for this coexistence has yet to be determined. Other tragic events were cerebral and ocular vasculitis, which manifested as coma and strabismus and highlight the importance of early diagnosis and effective immunosuppressive treatment.\u003c/p\u003e \u003cp\u003eMoreover, there are no suitable surgical or endovascular procedures for patients with TA and severe aortic insufficiency, severe calcification of the aorta (defined as a porcelain aorta). Indeed, manipulate friable and inflamed tissue increase the risk of valve detachment and anastomotic aneurysms, as well as manipulation of a porcelain aorta, which increases the risk of stroke and embolization.\u003c/p\u003e \u003cp\u003eDespite treatment with corticosteroids, infliximab, and mycophenolate mofetil, our patient\u0026rsquo;s rheumatologic symptoms continued to worsen. Fortunately, recent years have witnessed the emergence of new targeted biological agents for TA treatment. \u003csup\u003e\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u003c/sup\u003e Nonetheless, the increased infection risk precluded us from utilizing them.\u003c/p\u003e \u003cp\u003eThe last, but by no means the least, tragic event was our patient\u0026rsquo;s contracting COVID-19. Immunosuppressive agents, as the integral component of TA control, can increase susceptibility to infections. We commenced the latest universal treatment for COVID-19 promptly. Our patient, however, failed to respond to initial treatments, which indicates the lifesaving role of vaccination in patients with TA.\u003c/p\u003e"},{"header":"CONCLUSIONS","content":"\u003cp\u003eHere, we reported a novel case involving the coexistence of TA, severe aortic insufficiency, porcelain aorta, heart failure, cerebral and ocular vasculitis, and bronchiectasis with challenging management. Further investigations to determine the reasons for these associations will help devise new generations of treatments. In fact, our patient demonstrated the importance of early diagnosis, effective immunosuppressive treatment, new endovascular or surgical strategies and close follow-up.\u0026nbsp;\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003cstrong\u003e:\u0026nbsp;\u003c/strong\u003e Informed consent obtained from patient\u0026apos;s parents. Undoubtedly, we would provide the copy, if specifically requested by the journal.\u003c/p\u003e\n\u003cul\u003e\n \u003cli\u003eDeclarations of interest: None\u003c/li\u003e\n \u003cli\u003eDeclaration of Generative AI and AI-assisted technologies in the writing process: None\u003c/li\u003e\n \u003cli\u003eEthics approval and consent to participate:\u0026nbsp;Not applicable\u003c/li\u003e\n \u003cli\u003eConsent for publication: Not applicable\u003c/li\u003e\n \u003cli\u003eAvailability of data and materials: Not applicable\u003c/li\u003e\n \u003cli\u003eCompeting interests: Not applicable\u003c/li\u003e\n \u003cli\u003eFunding: Not applicable\u003c/li\u003e\n \u003cli\u003eAuthors\u0026apos; contributions: Not applicable\u003c/li\u003e\n \u003cli\u003eAcknowledgements: Not applicable\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials:\u0026nbsp;\u003c/strong\u003eAll data generated or analyzed during this study are included in this published article [and its supplementary information files].\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinical trial number:\u003c/strong\u003eThis article is just a case description without clinical trial. \u003cu\u003e(Number: 0)\u003c/u\u003e\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eFlorence A. Aeschlimann, Rae S. M. Yeung and Ronald M. Laxer, An Update on Childhood-Onset Takayasu Arteritis, 2022 Apr 13. doi: 10.3389/fped.2022.872313\u003c/li\u003e\n\u003cli\u003eRen Y, Du J, Guo X, Liu O, Liu W, Qi G, et al. Cardiac valvular involvement of Takayasu arteritis. \u003cem\u003eClin Rheumatol.\u003c/em\u003e (2021) 40:653\u0026ndash;60. doi: 10.1007/s10067-020-05290-2\u003c/li\u003e\n\u003cli\u003eLee GY, Jang SY, Ko SM, Kim EK, Lee SH, Han H, et al. Cardiovascular manifestations of Takayasu arteritis and their relationship to the disease activity: analysis of 204 Korean patients at a single center. Int J Cardiol. (2012) 159:14\u0026ndash;20. doi: 10.1016/j.ijcard.2011.01.094\u003c/li\u003e\n\u003cli\u003eFrancesca Regola, Martina Uzzo, Paola Toniati, Barbara Trezzi, Renato Alberto Sinico and Franco Franceschini1, Novel Therapies in Takayasu Arteritis, Front. Med., 12 January 2022, Sec. Rheumatology, Volume 8 \u0026ndash; 2021, https://doi.org/10.3389/fmed.2021.814075\u003c/li\u003e\n\u003cli\u003ePeter C Grayson, Cristina Ponte, Ravi Suppiah, Joanna C Robson, Katherine Bates Gibbons, Andrew Judge, Anthea Craven, Sara Khalid, Andrew Hutchings, Debashish Danda, Raashid A Luqmani, Richard A Watts, Peter A Merkel, 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis, BMJ journals, Ann Rheum Dis 2022;81:1654\u0026ndash;1660. doi:10.1136/ard-2022-223482\u003c/li\u003e\n\u003cli\u003eFrancesca Regola, Martina Uzzo, Paola Toniati, Barbara Trezzi, Renato Alberto Sinico and Franco Franceschini1, Novel Therapies in Takayasu Arteritis, Front. Med., 12 January 2022, Sec. Rheumatology, Volume 8 \u0026ndash; 2021, https://doi.org/10.3389/fmed.2021.814075\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-cardiovascular-disorders","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"bcar","sideBox":"Learn more about [BMC Cardiovascular Disorders](http://bmccardiovascdisord.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/bcar/default.aspx","title":"BMC Cardiovascular Disorders","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Case Report, Takayasu, Cardiovascular disease, Vasculitis","lastPublishedDoi":"10.21203/rs.3.rs-4442061/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4442061/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eA 17-year-old boy complaining of progressive dyspnea, fever, palpitations, a 22 mm Hg blood pressure difference between the 2 arms, and arm claudication. He had a history of psoriasis-like skin lesions and bronchiectasis. Echocardiography revealed a reduced left ventricular ejection fraction, severe eccentric aortic insufficiency, circumferential aortic wall thickening, and a dilated ascending aorta with severe atherosclerotic changes. Based on imaging findings, a TA diagnosis was suggested. During his follow-up, the patient developed strabismus, blurred vision, and right sixth cranial nerve paralysis and went into a deep coma. Unfortunately, after 6 months of treatment, he expired due to COVID-19 infection.\u003c/p\u003e","manuscriptTitle":"A Challenging case of Takayasu’s Arteritis in a young male with various manifestations and poor outcome","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-07-15 23:53:52","doi":"10.21203/rs.3.rs-4442061/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-09-19T09:02:28+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-09-15T14:02:50+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"105907479393923816747722397715990170237","date":"2024-09-14T03:35:45+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"139973090633331400675708305145466303788","date":"2024-09-08T14:44:13+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-08-04T05:33:00+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"28865172276459083431986628133715049068","date":"2024-07-14T11:11:24+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"2881647532118923425262861936947169681","date":"2024-07-08T07:35:52+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-06-20T04:42:45+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2024-06-18T17:12:47+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-06-18T09:27:55+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-06-18T09:26:55+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Cardiovascular Disorders","date":"2024-05-18T17:55:31+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-cardiovascular-disorders","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"bcar","sideBox":"Learn more about [BMC Cardiovascular Disorders](http://bmccardiovascdisord.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/bcar/default.aspx","title":"BMC Cardiovascular Disorders","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"d2da2f00-c7e7-464a-92f0-a4a7f4fd759d","owner":[],"postedDate":"July 15th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-11-18T16:00:37+00:00","versionOfRecord":{"articleIdentity":"rs-4442061","link":"https://doi.org/10.1186/s12872-024-04311-1","journal":{"identity":"bmc-cardiovascular-disorders","isVorOnly":false,"title":"BMC Cardiovascular Disorders"},"publishedOn":"2024-11-12 15:57:11","publishedOnDateReadable":"November 12th, 2024"},"versionCreatedAt":"2024-07-15 23:53:52","video":"","vorDoi":"10.1186/s12872-024-04311-1","vorDoiUrl":"https://doi.org/10.1186/s12872-024-04311-1","workflowStages":[]},"version":"v1","identity":"rs-4442061","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4442061","identity":"rs-4442061","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}