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IgG4-related spinal pachymeningitis.

Zhang Lu1, Liu Tongxi1, Luo Jie1

  • 1Department of Rheumatology and Immunology, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, 100029, Beijing, China.

Clinical Rheumatology
|November 17, 2015
PubMed
Summary

This study examines IgG4-related spinal pachymeningitis, a rare condition. Glucocorticoid therapy shows promise for treating this disease, particularly in older males affecting the cervical and thoracic vertebrae.

Keywords:
Cerebrospinal fluid IgG4 indexIgG4-related spinal pachymeningitisPathology

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Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • Immunoglobulin G4 (IgG4)-related disease is a systemic fibroinflammatory condition.
  • IgG4-related spinal pachymeningitis is a rare manifestation affecting the dura mater.

Observation:

  • A case of a 55-year-old male with extensive IgG4-related spinal pachymeningitis is presented.
  • Eight patients reported since 2009 predominantly affected older males, with lesions in cervical and thoracic vertebrae.
  • Common symptoms include limb/body numbness and weakness due to spinal cord compression.

Findings:

  • Elevated serum IgG4 levels were infrequent (1/5), but increased cerebrospinal fluid (CSF) IgG4 index was noted in some patients (3/3).
  • Histopathological findings are crucial for diagnosis.
  • All patients demonstrated a positive response to glucocorticoid therapy.

Implications:

  • IgG4-related spinal pachymeningitis is an orphan disease primarily impacting older males in the cervical and thoracic spine.
  • CSF IgG4 index may serve as a valuable diagnostic marker, as serum levels are often normal.
  • Glucocorticoid treatment appears effective for this condition, with pathological confirmation remaining the diagnostic gold standard.