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Abstract
Introduction: Vasculitis of the central nervous system (CNS) represents a heterogeneous set of inflammatory cerebrovascular pathologies, responsible for ischemia, infarctions and hemorrhages with broad neurological manifestations. Objective: This study aims to synthesize the most recent evidence on the etiology, diagnosis and therapeutic modalities of CNS vasculitis. Methodology: A systematic and qualitative review was performed in databases such as PubMed, MedlinePlus and SciELO, focusing on publications from the last fifteen years. Specific descriptors were applied together with Boolean operators to refine the search. The inclusion criteria aimed at articles and studies that directly addressed the etiological, diagnostic and therapeutic aspects of CNS vasculitis, excluding publications outside this scope or those that did not present textual integrity. Results and Discussion: CNS vasculitis can be categorized as primary, restricted to the nervous system, or secondary, associated with systemic pathologies. Genetic and environmental factors interact in the pathogenesis of these disorders. Diagnosis is challenging for clinicians due to symptomatic diversity and overlap with other neurological entities, requiring a combination of diagnostic methods, including laboratory tests, advanced neuroimaging, and histopathology via brain biopsy. Clinical management includes immunosuppression, primarily with corticosteroids and cyclophosphamide, in addition to emerging biological therapies for refractory cases. Final Considerations: Early recognition of CNS vasculitis is crucial but complex, requiring continuous diagnostic and therapeutic refinement. Future research should focus on the validation of diagnostic biomarkers and innovative therapies to improve clinical outcomes and quality of life for patients.
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