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Central Nervous System Vasculitis

Inflammation of blood vessels within the central nervous system. Primary vasculitis is usually caused by autoimmune or idiopathic factors, while secondary vasculitis is caused by existing disease process. Clinical manifestations are highly variable but include HEADACHE; SEIZURES; behavioral alterations; INTRACRANIAL HEMORRHAGES; TRANSIENT ISCHEMIC ATTACK; and BRAIN INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, pp856-61)
Also Known As:
Vasculitis, Central Nervous System; CNS Vasculitis; Central Nervous System Angiitis; Granulomatous Arteritis; Primary Central Nervous System Vasculitis; Vasculitis, CNS, Secondary; Angiitis, Cerebral; Angiitis, Granulomatous; Arteritis, Granulomatous; Arteritis, Postzoster; CNS Vasculitis, Primary; CNS Vasculitis, Secondary; Vasculitis, CNS; Vasculitis, Cerebral; Vasculitis, Primary CNS; Vasculitis, Secondary CNS; Angiitis, Central Nervous System; Cerebral Angiitis; Cerebral Vasculitis; Granulomatous Angiitis; Postzoster Arteritis; Primary CNS Vasculitis; Secondary CNS Vasculitis
Networked: 369 relevant articles (24 outcomes, 15 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Systemic Lupus Erythematosus (Libman-Sacks Disease)
2. Vasculitis (Vasculitides)
3. Hodgkin Disease (Hodgkin's Disease)
4. Fever (Fevers)
5. Stroke (Strokes)

Experts

1. Hunder, Gene G: 8 articles (04/2020 - 11/2007)
2. Huston, John: 8 articles (04/2020 - 11/2007)
3. Salvarani, Carlo: 8 articles (04/2020 - 11/2007)
4. Brown, Robert D: 7 articles (04/2020 - 11/2007)
5. Giannini, Caterina: 6 articles (04/2020 - 11/2007)
6. Christianson, Teresa J H: 5 articles (04/2020 - 11/2007)
7. Miller, Dylan V: 3 articles (08/2015 - 11/2007)
8. Kraemer, Markus: 2 articles (02/2022 - 09/2017)
9. Iglesias, Estibaliz: 2 articles (01/2022 - 01/2019)
10. Schaper, Jörg: 2 articles (01/2022 - 03/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Central Nervous System Vasculitis:
1. SteroidsIBA
2. Cyclophosphamide (Cytoxan)FDA LinkGeneric
3. Adrenal Cortex Hormones (Corticosteroids)IBA
4. MethylprednisoloneFDA LinkGeneric
5. Immunosuppressive Agents (Immunosuppressants)IBA
01/01/2004 - "The potential for response of the process to antimicrobials and/or immunosuppressants, and the potential for devastating consequences if the process is left untreated, has heightened the urgency in recognizing CNS vasculitis. "
09/01/2023 - "Encephalopathy in DM may occur for a number of reasons, such as cerebral vasculitis and hypoperfusion/hypertensive encephalopathy, but mostly as a consequence of immunosuppressant treatment. "
01/01/2018 - "Methods- We assessed the rate of prolonged remission (defined by the absence of relapse at ≥12 months after diagnosis) and the functional status at last follow-up in patients with primary central nervous system vasculitis included in the French cohort, who achieved a first remission according to the 3 main groups of treatments administered: glucocorticoids only (group 1); induction treatment with glucocorticoids and an immunosuppressant, but no maintenance (group 2); and combined treatment with glucocorticoids and an immunosuppressant for induction followed by maintenance therapy (group 3). "
01/01/2018 - "Conclusions- This study suggests that maintenance therapy with an immunosuppressant combined with glucocorticoids lead to the best long-term clinical and functional outcomes in patients with primary central nervous system vasculitis after having achieved remission with either glucocorticoids alone or in combination with another immunosuppressant."
01/01/2009 - "Standard therapy of cerebral vasculitis includes corticosteroids often combined with immunosuppressants."
6. Rituximab (Mabthera)FDA Link
7. GlucocorticoidsIBA
8. Intravenous Immunoglobulins (IVIG)FDA Link
9. Anti-Bacterial Agents (Antibiotics)IBA
10. Antigen-Antibody Complex (Immune Complex)IBA

Therapies and Procedures

1. Therapeutics
2. Aftercare (After-Treatment)
3. Plasmapheresis
4. Drug Therapy (Chemotherapy)
5. Hemispherectomy