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[Central nervous system involvement in connective tissue diseases].

Shunsei Hirohata1

  • 1Department of Internal Medicine, Teikyo University School of Medicine.

Nihon Rinsho Men'Eki Gakkai Kaishi = Japanese Journal of Clinical Immunology
|August 5, 2004
PubMed
Summary

Neuropsychiatric lupus (CNS lupus) involves elevated immune markers in cerebrospinal fluid (CSF). Specific antibodies like anti-neuronal (anti-N) may indicate CNS lupus psychosis pathogenesis.

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Effect of Infliximab on Chronic Progressive Neuro-Behçet's Disease: Influence of the Timing of Introduction on the Patient Outcome.

Internal medicine (Tokyo, Japan)·2023

Area of Science:

  • Neurology
  • Immunology
  • Rheumatology

Context:

  • Neuropsychiatric manifestations are common in systemic lupus erythematosus (CNS lupus).
  • Central nervous system (CNS) involvement occurs in Behçet's disease (neuro-Behçet's syndrome) and ANCA-related vasculitis.
  • Elevated immune markers in cerebrospinal fluid (CSF) are observed in CNS lupus.

Purpose:

  • To investigate the role of immune system activation within the CNS in the pathogenesis of CNS lupus.
  • To characterize CNS involvement in Behçet's disease and ANCA-related vasculitis.
  • To identify specific biomarkers associated with CNS lupus manifestations.

Summary:

  • CSF IgM, IgA, IgG indexes, and IL-6 activities are elevated in CNS lupus patients.
  • Anti-ribosomal P antibody (anti-P) in sera and anti-neuronal antibody (anti-N) in CSF are elevated in lupus psychosis.

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  • Neuro-Behçet's syndrome presents with acute meningoencephalitis or chronic progressive dementia, with elevated CSF IL-6 in the chronic form.
  • Pachymeningitis in ANCA-related vasculitis is associated with P-ANCA and C-ANCA.

    • Impact:

    • Findings suggest CNS immune activation, potentially involving CSF anti-N, is crucial for CNS lupus pathogenesis, including psychosis.
    • Understanding these immune mechanisms may lead to targeted therapies for CNS lupus and related neurological autoimmune disorders.
    • Differentiates neuro-Behçet's syndrome subtypes and treatment responses, highlighting methotrexate for chronic progressive forms.
    • Establishes associations between specific ANCA types and pachymeningitis.