Cerebral Vasculitis in Systemic Lupus Erythematosus (SLE) : Case Report and Literature Review

Cerebral Vasculitis in Systemic Lupus Erythematosus (SLE) : Case Report and Literature Review | 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 Research Article Cerebral Vasculitis in Systemic Lupus Erythematosus (SLE) : Case Report and Literature Review Abderrazzak AJERTIL, Najat KABBAJ, Abdeljalil EL QUESSAR This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-5363740/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Cerebral vasculitis, though rare, represents a serious complication of systemic lupus erythematosus (SLE) and poses numerous challenges in terms of management due to its potential for severe neurological consequences and often poor prognosis. We report the case of a 32-year-old man with SLE who presented with three seizure episodes and cognitive deterioration. Neurological examinations, laboratory analyses, and imaging findings led to a diagnosis of cerebral vasculitis secondary to SLE. Despite intensive immunosuppressive treatment, the patient’s neurological condition deteriorated, resulting in multiorgan failure. Ultimately, the patient died due to multiorgan failure linked to severe central nervous system vasculitis and associated complications. This case highlights the importance of early detection and aggressive treatment of cerebral vasculitis in the context of SLE. systemic lupus erythematosus (SLE) cerebral vasculitis neurological complications multiorgan failure immunological dysfunction Figures Figure 1 Introduction Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by immune system dysfunction, leading to inflammation and damage across various organs and systems in the body. While SLE primarily affects women of childbearing age, clinical manifestations can vary widely, from mild symptoms to life-threatening complications [1]. About half of individuals with SLE experience neurological symptoms at some point during their disease, with varying severity and clinical forms [2]. Cerebral vasculitis, though uncommon, is one of the most severe neurological complications of SLE, associated with high morbidity and mortality rates [3]. SLE affects multiple organ systems, including muscles as well as the peripheral and central nervous systems. While central nervous system (CNS) involvement is frequent and leads to a variety of neuropsychiatric symptoms in SLE patients, cerebral vasculitis remains a rare manifestation, as confirmed by post-mortem examinations showing a low incidence of this condition. In the context of SLE, vasculitis is characterized by inflammation of cerebral blood vessels, disrupting blood flow and resulting in ischemia and tissue damage. Clinically, this condition can cause seizures, focal neurological deficits, cognitive impairment, and altered consciousness [4]. Diagnosing cerebral vasculitis in SLE patients often requires a thorough clinical evaluation, including laboratory tests and neuroimaging studies. Laboratory results may reveal positive antinuclear antibodies (ANA), elevated inflammatory markers, and evidence of multi-organ dysfunction [5]. Imaging studies, such as MRI, play a key role in detecting abnormalities associated with cerebral vasculitis, including white matter lesions, infarcts, and hemorrhages [6]. Treatment generally relies on powerful immunosuppressants aimed at reducing inflammation and controlling the underlying autoimmune process. Corticosteroids, cyclophosphamide, and other immunomodulatory agents are commonly used to prevent the worsening of neurological symptoms [7]. Despite advancements in the management of SLE and its complications, cerebral vasculitis remains challenging to treat and is often associated with a poor prognosis. Early identification, prompt intervention, and continuous monitoring are crucial to improving outcomes for affected patients. In this context, we present the case of a 32-year-old man with SLE who developed cerebral vasculitis, highlighting clinical signs, diagnostic challenges, and therapeutic approaches for this rare but serious complication. Case Presentation A 32-year-old patient presented to the emergency room with seizure episodes characterized by abnormal movements of the upper and lower limbs, accompanied by jaw clenching and severe headaches. A year prior, he had exhibited symptoms such as arthralgia, fatigue, skin rashes, photosensitivity, and mouth ulcers, as well as two seizure episodes, with no significant family history of autoimmune or neurological disorders. Upon physical examination in the emergency room, his blood pressure was 150/85 mm Hg, pulse was 100 bpm, and oxygen saturation was 96%. Neurological evaluation revealed generalized tonic-clonic seizures, limited spontaneous movement, and unreactive pupils. Deep tendon reflexes were diminished or absent. Systemic examinations of the cardiovascular, respiratory, and gastrointestinal systems showed no abnormalities. Additionally, a malar erythematous rash sparing the nasolabial folds, consistent with SLE, was observed. Diagnostic assessment and treatment The patient underwent a comprehensive series of laboratory and imaging tests, revealing significant findings. Notably, a positive anti-Smith antibody (Anti-Sm), highly specific for SLE, and an antinuclear antibody (ANA), the most sensitive for this diagnosis, were identified. The tests also showed reduced complement levels (C4 at 7 mg/dL and C3 at 57 mg/dL). Iron deficiency anemia was detected with a hemoglobin level of 9 g/L, and oral iron protein succinylate was prescribed for one month; however, no notable improvement was observed. A blood smear revealed a high presence of spherocytes, suggesting an autoimmune hemolytic process, necessitating the initiation of corticosteroid therapy. The patient also had an elevated serum creatinine level of 32 mg/L, indicating impaired renal function. Finally, cerebrospinal fluid (CSF) analysis showed elevated protein levels and pleocytosis, indicating central nervous system (CNS) inflammation. Radiological examinations included chest X-ray, abdominal and pelvic ultrasound, and brain MRI. The chest X-ray was normal, the ultrasound revealed cortico-medullary dedifferentiation, suggesting chronic kidney disease. The brain MRI, performed on a 1.5 Tesla MRI machine, showed T2/FLAIR hyperintensities in the cerebral cortex and juxtacortical and subcortical white matter of both cerebral hemispheres as well as in the right cerebellar hemisphere. Additionally, multiple small cortical and subcortical ischemic foci were visible on DWI/FLAIR sequences. The susceptibility-weighted imaging (SWI) sequence revealed diffuse petechial hemorrhages in the bilateral cerebral lobes, primarily involving the cerebral cortex and subcortical white matter, as shown in Figure 1. The patient received treatment including corticosteroids (Methylprednisolone 1g IV) to control inflammation, antiepileptics to control seizures, and cyclophosphamide to address the ongoing autoimmune process. Despite aggressive medical intervention for SLE, the patient’s neurological status continued to deteriorate. He developed respiratory failure with pulmonary edema, followed by coma, requiring respiratory support. Despite all supportive care provided, the patient subsequently developed multiorgan failure, leading to his death. Discussion Cerebral vasculitis is a rare but serious complication of systemic lupus erythematosus (SLE), involving inflammation of the cerebral blood vessels and presenting a significant risk of severe neurological complications, along with a poor prognosis [8]. In our patient, he showed various neurological symptoms such as seizures, altered cognitive functions, and unresponsiveness, indicating central nervous system (CNS) involvement related to SLE. The presence of a malar rash, positive anti-Smith (Anti-Sm) and antinuclear (ANA) antibodies, along with other laboratory findings consistent with SLE, supported this diagnosis. Moreover, imaging tests revealed signs of cerebral vasculitis, including T2/FLAIR hyperintensities and petechial hemorrhages in the cerebral parenchyma [9]. To exclude secondary infections, contrast-enhanced MRI is necessary. In addition to standard MRI, vascular wall MRI (VW-MRI) can help differentiate vascular narrowing related to intracranial atherosclerotic plaque, reversible cerebral vasoconstriction syndrome, dissection, Moyamoya disease, as well as vasculitis, which presents as enhancement of the affected arterial wall. The treatment of cerebral vasculitis in the context of SLE generally relies on an aggressive immunosuppressive protocol aimed at controlling the underlying autoimmune process and reducing inflammation. In this case, the patient received a combination of corticosteroids and cyclophosphamide to limit disease activity and prevent neurological worsening [10]. Despite these interventions, the patient’s neurological condition continued to deteriorate, illustrating the challenges in managing this SLE complication. The onset of respiratory failure and multiorgan dysfunction further complicated the situation. Despite intensive supportive care, including respiratory assistance and hemodynamic management, the patient ultimately succumbed to multiorgan failure, attributable to CNS vasculitis and its complications [11]. Conclusion In conclusion, this case highlights the major challenges posed by cerebral vasculitis in the context of systemic lupus erythematosus (SLE). Despite aggressive management, including immunosuppressive therapy and supportive care, the patient's neurological condition worsened, ultimately leading to multiorgan failure and a tragic outcome. This situation underscores the limitations of current therapeutic approaches and the urgent need to pursue more effective strategies for treating cerebral vasculitis in SLE. It also emphasizes the importance of early detection and rapid treatment to improve clinical outcomes. Finally, the complexity and severity of this condition necessitate a multidisciplinary approach, involving rheumatologists, neurologists, and intensive care specialists, to optimize care and reduce the potentially devastating impact of this disease. Declarations I hereby confirm that consent has been obtained from the patient and all relevant physicians for the participation and publication of this case report. Conflicts of Interest The authors declare no conflicts of interest. Author Contributions Dr. AJERTIL is the primary author, Professor Kabbaj and Professor Abdeljalil EL QUESSAR contributed to the development of this work by providing her expertise in writing. References Bertsias GK, Ioannidis JP, Aringer M, et al.: EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs. Ann. Rheum. Dis. 2010;69(12):2074–2082. 10.1136/ard.2010.130476 Hanly JG, Urowitz MB, Sanchez-Guerrero J, et al.: Neuropsychiatric events at the time of diagnosis of systemic lupus erythematosus: an international inception cohort study. Arthritis Rheum. 2007;56(1):265–273. 10.1002/art.22305 Zhang X, Dong Y, Tang F, et al.: Central nervous system involvement in systemic lupus erythematosus. Zhonghua Nei Ke Za Zhi. 1999;38(10):681–684. Govoni M, Bortoluzzi A, Padovan M, et al.: The diagnosis and clinical management of the neuropsychiatric manifestations of lupus. J. Autoimmun. 2016;74:41–72. 10.1016/j.jaut.2016.06.013 Thirunavukkarasu B, Gupta K, Nada R, et al.: Neuropathological spectrum in systemic lupus erythematosus: A single institute autopsy experience. J. Neuroimmunol. 2021;353:577518. 10.1016/j.jneuroim.2021.577518 Emerson JS, Gruenewald SM, Gomes L, et al.: The conundrum of neuropsychiatric systemic lupus erythematosus: Current and novel approaches to diagnosis. Front. Neurol. 2023;14:1111769. 10.3389/fneur.2023.1111769 Jennette JC, Falk RJ, Bacon PA, et al.: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. 10.1002/art.37715 Bertsias GK, Tektonidou M, Amoura Z, et al.: Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann. Rheum. Dis. 2012;71(11):1771–1782. 10.1136/annrheumdis-2012-201940 Fanouriakis A, Kostopoulou M, Alunno A, et al.: 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann. Rheum. Dis. 2019;78(6):736–745. 10.1136/annrheumdis-2019-215089 Lassere MN, Johnson KR, Boers M, et al.: Definitions and validation criteria for biomarkers and surrogate endpoints: development and testing of a quantitative hierarchical levels of evidence schema. J. Rheumatol. 2007;34(3):607–615. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted 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. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. 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-5363740","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":372654250,"identity":"8460e1a7-86fe-4738-9ae2-b8edf5dae204","order_by":0,"name":"Abderrazzak AJERTIL","email":"data:image/png;base64,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","orcid":"","institution":"Cheikh Zayd International University Hospital, UIASS","correspondingAuthor":true,"prefix":"","firstName":"Abderrazzak","middleName":"","lastName":"AJERTIL","suffix":""},{"id":372654251,"identity":"5c85176a-b0aa-4f5f-a649-23bbd911536e","order_by":1,"name":"Najat KABBAJ","email":"","orcid":"","institution":"Cheikh Zayd International University Hospital, UIASS","correspondingAuthor":false,"prefix":"","firstName":"Najat","middleName":"","lastName":"KABBAJ","suffix":""},{"id":372654252,"identity":"a3763c54-1006-4c6d-b08f-3b8d4df6d2a8","order_by":2,"name":"Abdeljalil EL QUESSAR","email":"","orcid":"","institution":"Cheikh Zayd International University Hospital, UIASS","correspondingAuthor":false,"prefix":"","firstName":"Abdeljalil","middleName":"EL","lastName":"QUESSAR","suffix":""}],"badges":[],"createdAt":"2024-10-30 23:38:11","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-5363740/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-5363740/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":68354567,"identity":"aba05f3f-a4ac-49aa-ace5-0019800989f1","added_by":"auto","created_at":"2024-11-06 11:08:51","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":216457,"visible":true,"origin":"","legend":"\u003cp\u003eA: Axial slices from the Diffusion sequence. B: Axial slices from the FLAIR sequence. C: SWI sequence. D: 3D TOF sequence. These sequences show multiple cortico-subcortical areas affecting both the posterior fossa and the supratentorial level, appearing on FLAIR with punctate diffusion restriction in some areas, without ADC drop. Presence of cortico-subcortical microbleeds, visible in the SWI sequence. The 3D TOF Angio-MRI sequence does not show any abnormalities in the Circle of Willis.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-5363740/v1/2c3815194cbc3a03496f9298.png"},{"id":68355260,"identity":"10c37c56-0bac-4bc8-bfbe-a2aac2f61dd5","added_by":"auto","created_at":"2024-11-06 11:16:46","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":433034,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-5363740/v1/db5012db-0c13-4328-b4db-6f63e315a1a5.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Cerebral Vasculitis in Systemic Lupus Erythematosus (SLE) : Case Report and Literature Review","fulltext":[{"header":"Introduction","content":"\u003cp\u003eSystemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by immune system dysfunction, leading to inflammation and damage across various organs and systems in the body. While SLE primarily affects women of childbearing age, clinical manifestations can vary widely, from mild symptoms to life-threatening complications [1]. About half of individuals with SLE experience neurological symptoms at some point during their disease, with varying severity and clinical forms [2]. Cerebral vasculitis, though uncommon, is one of the most severe neurological complications of SLE, associated with high morbidity and mortality rates [3]. SLE affects multiple organ systems, including muscles as well as the peripheral and central nervous systems. While central nervous system (CNS) involvement is frequent and leads to a variety of neuropsychiatric symptoms in SLE patients, cerebral vasculitis remains a rare manifestation, as confirmed by post-mortem examinations showing a low incidence of this condition. In the context of SLE, vasculitis is characterized by inflammation of cerebral blood vessels, disrupting blood flow and resulting in ischemia and tissue damage. Clinically, this condition can cause seizures, focal neurological deficits, cognitive impairment, and altered consciousness [4]. Diagnosing cerebral vasculitis in SLE patients often requires a thorough clinical evaluation, including laboratory tests and neuroimaging studies. Laboratory results may reveal positive antinuclear antibodies (ANA), elevated inflammatory markers, and evidence of multi-organ dysfunction [5]. Imaging studies, such as MRI, play a key role in detecting abnormalities associated with cerebral vasculitis, including white matter lesions, infarcts, and hemorrhages [6]. Treatment generally relies on powerful immunosuppressants aimed at reducing inflammation and controlling the underlying autoimmune process. Corticosteroids, cyclophosphamide, and other immunomodulatory agents are commonly used to prevent the worsening of neurological symptoms [7]. Despite advancements in the management of SLE and its complications, cerebral vasculitis remains challenging to treat and is often associated with a poor prognosis. Early identification, prompt intervention, and continuous monitoring are crucial to improving outcomes for affected patients. In this context, we present the case of a 32-year-old man with SLE who developed cerebral vasculitis, highlighting clinical signs, diagnostic challenges, and therapeutic approaches for this rare but serious complication.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 32-year-old patient presented to the emergency room with seizure episodes characterized by abnormal movements of the upper and lower limbs, accompanied by jaw clenching and severe headaches. A year prior, he had exhibited symptoms such as arthralgia, fatigue, skin rashes, photosensitivity, and mouth ulcers, as well as two seizure episodes, with no significant family history of autoimmune or neurological disorders. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003eUpon physical examination in the emergency room, his blood pressure was 150/85 mm Hg, pulse was 100 bpm, and oxygen saturation was 96%. Neurological evaluation revealed generalized tonic-clonic seizures, limited spontaneous movement, and unreactive pupils. Deep tendon reflexes were diminished or absent. Systemic examinations of the cardiovascular, respiratory, and gastrointestinal systems showed no abnormalities. Additionally, a malar erythematous rash sparing the nasolabial folds, consistent with SLE, was observed.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eDiagnostic assessment and treatment\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe patient underwent a comprehensive series of laboratory and imaging tests, revealing significant findings. Notably, a positive anti-Smith antibody (Anti-Sm), highly specific for SLE, and an antinuclear antibody (ANA), the most sensitive for this diagnosis, were identified. The tests also showed reduced complement levels (C4 at 7 mg/dL and C3 at 57 mg/dL). Iron deficiency anemia was detected with a hemoglobin level of 9 g/L, and oral iron protein succinylate was prescribed for one month; however, no notable improvement was observed. A blood smear revealed a high presence of spherocytes, suggesting an autoimmune hemolytic process, necessitating the initiation of corticosteroid therapy.\u003c/p\u003e\n\u003cp\u003eThe patient also had an elevated serum creatinine level of 32 mg/L, indicating impaired renal function. Finally, cerebrospinal fluid (CSF) analysis showed elevated protein levels and pleocytosis, indicating central nervous system (CNS) inflammation.\u003c/p\u003e\n\u003cp\u003eRadiological examinations included chest X-ray, abdominal and pelvic ultrasound, and brain MRI. The chest X-ray was normal, the ultrasound revealed cortico-medullary dedifferentiation, suggesting chronic kidney disease. The brain MRI, performed on a 1.5 Tesla MRI machine, showed T2/FLAIR hyperintensities in the cerebral cortex and juxtacortical and subcortical white matter of both cerebral hemispheres as well as in the right cerebellar hemisphere. Additionally, multiple small cortical and subcortical ischemic foci were visible on DWI/FLAIR sequences. The susceptibility-weighted imaging (SWI) sequence revealed diffuse petechial hemorrhages in the bilateral cerebral lobes, primarily involving the cerebral cortex and subcortical white matter, as shown in Figure 1.\u003c/p\u003e\n\u003cp\u003eThe patient received treatment including corticosteroids (Methylprednisolone 1g IV) to control inflammation, antiepileptics to control seizures, and cyclophosphamide to address the ongoing autoimmune process. Despite aggressive medical intervention for SLE, the patient\u0026rsquo;s neurological status continued to deteriorate. He developed respiratory failure with pulmonary edema, followed by coma, requiring respiratory support. Despite all supportive care provided, the patient subsequently developed multiorgan failure, leading to his death.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eCerebral vasculitis is a rare but serious complication of systemic lupus erythematosus (SLE), involving inflammation of the cerebral blood vessels and presenting a significant risk of severe neurological complications, along with a poor prognosis [8]. In our patient, he showed various neurological symptoms such as seizures, altered cognitive functions, and unresponsiveness, indicating central nervous system (CNS) involvement related to SLE. The presence of a malar rash, positive anti-Smith (Anti-Sm) and antinuclear (ANA) antibodies, along with other laboratory findings consistent with SLE, supported this diagnosis. Moreover, imaging tests revealed signs of cerebral vasculitis, including T2/FLAIR hyperintensities and petechial hemorrhages in the cerebral parenchyma [9]. To exclude secondary infections, contrast-enhanced MRI is necessary. In addition to standard MRI, vascular wall MRI (VW-MRI) can help differentiate vascular narrowing related to intracranial atherosclerotic plaque, reversible cerebral vasoconstriction syndrome, dissection, Moyamoya disease, as well as vasculitis, which presents as enhancement of the affected arterial wall.\u003c/p\u003e\n\u003cp\u003eThe treatment of cerebral vasculitis in the context of SLE generally relies on an aggressive immunosuppressive protocol aimed at controlling the underlying autoimmune process and reducing inflammation. In this case, the patient received a combination of corticosteroids and cyclophosphamide to limit disease activity and prevent neurological worsening [10]. Despite these interventions, the patient\u0026rsquo;s neurological condition continued to deteriorate, illustrating the challenges in managing this SLE complication. The onset of respiratory failure and multiorgan dysfunction further complicated the situation. Despite intensive supportive care, including respiratory assistance and hemodynamic management, the patient ultimately succumbed to multiorgan failure, attributable to CNS vasculitis and its complications [11].\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eIn conclusion, this case highlights the major challenges posed by cerebral vasculitis in the context of systemic lupus erythematosus (SLE). Despite aggressive management, including immunosuppressive therapy and supportive care, the patient\u0026apos;s neurological condition worsened, ultimately leading to multiorgan failure and a tragic outcome. This situation underscores the limitations of current therapeutic approaches and the urgent need to pursue more effective strategies for treating cerebral vasculitis in SLE. It also emphasizes the importance of early detection and rapid treatment to improve clinical outcomes. Finally, the complexity and severity of this condition necessitate a multidisciplinary approach, involving rheumatologists, neurologists, and intensive care specialists, to optimize care and reduce the potentially devastating impact of this disease.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eI hereby confirm that consent has been obtained from the patient and all relevant physicians for the participation and publication of this case report.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflicts of Interest\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no conflicts of interest.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor Contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eDr. AJERTIL is the primary author, Professor Kabbaj and Professor Abdeljalil EL QUESSAR contributed to the development of this work by providing her expertise in writing.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eBertsias GK, Ioannidis JP, Aringer M, et al.: EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs. Ann. Rheum. Dis. 2010;69(12):2074\u0026ndash;2082. 10.1136/ard.2010.130476 \u003c/li\u003e\n\u003cli\u003eHanly JG, Urowitz MB, Sanchez-Guerrero J, et al.: Neuropsychiatric events at the time of diagnosis of systemic lupus erythematosus: an international inception cohort study. Arthritis Rheum. 2007;56(1):265\u0026ndash;273. 10.1002/art.22305 \u003c/li\u003e\n\u003cli\u003eZhang X, Dong Y, Tang F, et al.: Central nervous system involvement in systemic lupus erythematosus. Zhonghua Nei Ke Za Zhi. 1999;38(10):681\u0026ndash;684. \u003c/li\u003e\n\u003cli\u003eGovoni M, Bortoluzzi A, Padovan M, et al.: The diagnosis and clinical management of the neuropsychiatric manifestations of lupus. J. Autoimmun. 2016;74:41\u0026ndash;72. 10.1016/j.jaut.2016.06.013 \u003c/li\u003e\n\u003cli\u003eThirunavukkarasu B, Gupta K, Nada R, et al.: Neuropathological spectrum in systemic lupus erythematosus: A single institute autopsy experience. J. Neuroimmunol. 2021;353:577518. 10.1016/j.jneuroim.2021.577518 \u003c/li\u003e\n\u003cli\u003eEmerson JS, Gruenewald SM, Gomes L, et al.: The conundrum of neuropsychiatric systemic lupus erythematosus: Current and novel approaches to diagnosis. Front. Neurol. 2023;14:1111769. 10.3389/fneur.2023.1111769 \u003c/li\u003e\n\u003cli\u003eJennette JC, Falk RJ, Bacon PA, et al.: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1\u0026ndash;11. 10.1002/art.37715 \u003c/li\u003e\n\u003cli\u003eBertsias GK, Tektonidou M, Amoura Z, et al.: Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann. Rheum. Dis. 2012;71(11):1771\u0026ndash;1782. 10.1136/annrheumdis-2012-201940 \u003c/li\u003e\n\u003cli\u003eFanouriakis A, Kostopoulou M, Alunno A, et al.: 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann. Rheum. Dis. 2019;78(6):736\u0026ndash;745. 10.1136/annrheumdis-2019-215089 \u003c/li\u003e\n\u003cli\u003eLassere MN, Johnson KR, Boers M, et al.: Definitions and validation criteria for biomarkers and surrogate endpoints: development and testing of a quantitative hierarchical levels of evidence schema. J. Rheumatol. 2007;34(3):607\u0026ndash;615. \u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"systemic lupus erythematosus (SLE), cerebral vasculitis, neurological complications, multiorgan failure, immunological dysfunction","lastPublishedDoi":"10.21203/rs.3.rs-5363740/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-5363740/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"Cerebral vasculitis, though rare, represents a serious complication of systemic lupus erythematosus (SLE) and poses numerous challenges in terms of management due to its potential for severe neurological consequences and often poor prognosis. We report the case of a 32-year-old man with SLE who presented with three seizure episodes and cognitive deterioration. Neurological examinations, laboratory analyses, and imaging findings led to a diagnosis of cerebral vasculitis secondary to SLE. Despite intensive immunosuppressive treatment, the patient’s neurological condition deteriorated, resulting in multiorgan failure. Ultimately, the patient died due to multiorgan failure linked to severe central nervous system vasculitis and associated complications. This case highlights the importance of early detection and aggressive treatment of cerebral vasculitis in the context of SLE.","manuscriptTitle":"Cerebral Vasculitis in Systemic Lupus Erythematosus (SLE) : Case Report and Literature Review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-11-06 11:08:37","doi":"10.21203/rs.3.rs-5363740/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"d10da51f-6217-4d5d-8904-ddb7a4ee880d","owner":[],"postedDate":"November 6th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-11-06T11:08:37+00:00","versionOfRecord":[],"versionCreatedAt":"2024-11-06 11:08:37","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-5363740","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-5363740","identity":"rs-5363740","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}