Neurology Rare Disease Society2024EN
Clinical Spectrum and One-Year Outcomes of CNS Vasculitis
Zusammenfassung
Clinical Spectrum and One-Year Outcomes of Central Nervous System Vasculitis Secondary to Systemic Autoimmune Disorders: A Retrospective Cohort Study
ABSTRACT
Abstract
Introduction
Central nervous system (CNS) vasculitis (CNSV) is a rare but serious complication of systemic autoimmune disorders, including systemic lupus erythematosus, antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, rheumatoid arthritis, polyarteritis nodosa, Sjögren’s syndrome, and mixed connective tissue di
Kerninformationen
Very high antibody titers and elevated C-reactive protein (CRP) correlated with poor outcomes, while moderate titers did not. MRI abnormalities were present in 135 patients (90%), most often multiple-territory infarcts in 55 patients (36.7%), lacunar infarcts in 40 patients (26.7%), and white matter...
Clinical features, laboratory data, and neuroimaging findings were extracted from records using a structured proforma. Treatments were categorized as corticosteroids alone, steroids plus conventional immunosuppressants, or steroids plus biologics. Patients were followed for 12 months, with functiona...
In secondary CNSV, systemic autoimmunity such as lupus, antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, or rheumatoid arthritis leads to vascular inflammation in the CNS, causing ischemic or hemorrhagic injury, seizures, encephalopathy, and other neurological deficits. This disor...
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