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Clinical and prognostic profiles in immune-mediated hypertrophic meningitis: a retrospective analysis of 92 cases

Yifei Wang1, Yuyan Yang2, Xuemei Guo3

  • 1Department of Rheumatology and Clinical Immunology, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuai-Fu-Yuan, Dongcheng District, 100730, Beijing, China.

Clinical Rheumatology
|June 12, 2025
PubMed
Abstract

Insights

Immune-mediated hypertrophic pachymeningitis (HP) presents similarly across its main causes: ANCA-related, idiopathic, and IgG4-related disease. These diverse etiologies share comparable clinical features and long-term outcomes, suggesting a unified treatment approach may be beneficial.

Area of Science:

  • Neurology
  • Immunology
  • Rheumatology

Background:

  • Immune-mediated hypertrophic pachymeningitis (HP) is a rare inflammatory condition causing diverse neurological symptoms.
  • Its rarity and varied causes present diagnostic challenges, with limited comparative studies on different etiologies.
  • Understanding clinical and prognostic differences is crucial for effective management.

Purpose of the Study:

  • To analyze and compare clinical characteristics, treatment strategies, and outcomes of immune-mediated HP patients.
  • To investigate the predominant etiologies of HP in a Chinese cohort.
  • To explore similarities and differences among antineutrophil cytoplasmic antibody (ANCA)-related HP, idiopathic HP, and IgG4-related disease (IgG4-RD).

Main Methods:

  • A retrospective study of 92 immune-mediated HP cases from December 2003 to September 2021 at a single center in China.
  • Evaluation of neurological symptoms, systemic involvement, imaging findings, and laboratory results.
  • Comparison of clinical features, treatment approaches, and outcomes across ANCA-related HP, idiopathic HP, and IgG4-RD.

Main Results:

  • Antineutrophil cytoplasmic antibody (ANCA)-related HP (45.6%) was the most common cause, followed by idiopathic HP (30.4%) and IgG4-related disease (13.0%).
  • Despite differing etiologies, similar patterns of cranial nerve and brain region involvement were observed.
  • The most common treatment involved high-dose glucocorticoids with immunosuppressants, and no significant differences in relapse-free survival were found among the subgroups after a mean follow-up of 4.1 years.

Conclusions:

  • ANCA-related HP, idiopathic HP, and IgG4-RD HP are the primary causes of immune-mediated HP in this Chinese cohort.
  • These etiologies demonstrate similar clinical manifestations, imaging findings, and long-term prognoses.
  • A standardized management strategy may be effective for HP patients, irrespective of their underlying etiology.
Keywords:
Immunoglobulin G4-related diseaseMeningitisPrognosisVasculitis

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