Hypertrophic pachymeningitis in ANCA-associated vasculitis: Clinical and immunopathological features and insights.

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  • Author(s): Shimojima Y;Shimojima Y; Sekijima Y; Sekijima Y
  • Source:
    Autoimmunity reviews [Autoimmun Rev] 2023 Jun; Vol. 22 (6), pp. 103338. Date of Electronic Publication: 2023 Apr 14.
  • Publication Type:
    Journal Article; Review
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128967 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-0183 (Electronic) Linking ISSN: 15689972 NLM ISO Abbreviation: Autoimmun Rev Subsets: MEDLINE
    • Publication Information:
      Original Publication: Amsterdam ; New York : Elsevier, c2002-
    • Subject Terms:
      Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis*/complications ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis*/drug therapy ; Meningitis*/complications ; Meningitis*/drug therapy ; Granulomatosis with Polyangiitis*/complications ; Granulomatosis with Polyangiitis*/drug therapy; Humans ; Antibodies, Antineutrophil Cytoplasmic ; Immunosuppressive Agents/therapeutic use ; Cyclophosphamide/therapeutic use ; Peroxidase
    • Abstract:
      Hypertrophic pachymeningitis (HP) is an inflammatory disorder characterized by intracranial and spinal thickened dura mater, leading to several neurological manifestations including headaches, cranial neuropathies, seizures, and sensorimotor disorders. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a crucial disease that is implicated in the development of immune-mediated HP. HP is observed throughout the clinical course of AAV, and 3%-4% of patients with AAV experience HP as the initial clinical episode. However, patients with ANCA-related HP are unclassifiable in the classification criteria of AAV when HP is the only manifestation, suggesting that ANCA-related HP can be identified as a central nervous system-limited type of AAV. Among patients with AAV, those who develop HP have predominantly been classified as having granulomatosis with polyangiitis (GPA). Myeloperoxidase-ANCA positivity has been more frequently demonstrated than proteinase 3-ANCA positivity in patients with ANCA-related HP. The ear, nose, and throat manifestations, such as otitis media, sinusitis, and mastoiditis, as well as mucous membranes/eyes manifestations including sudden visual loss, are robustly associated with HP in AAV. The histology of thickened dura mater tissues includes fibrotic changes and infiltration of several immunocompetent cells, but the typical findings of GPA, such as granulomatous inflammation with necrotizing vasculitis, are not observed in all patients with ANCA-related HP. Corticosteroids are the first-line therapy for ANCA-related HP, while the concomitant use of immunosuppressive agents including cyclophosphamide, methotrexate, and mycophenolate mofetil, is an ideal strategy for achieving remission. Rituximab is a useful agent in refractory ANCA-related HP.
      Competing Interests: Declaration of Competing Interest The authors declared that they have no financial or personal conflicts of interest.
      (Copyright © 2023 Elsevier B.V. All rights reserved.)
    • Contributed Indexing:
      Keywords: Antineutrophil cytoplasmic antibody; Antineutrophil cytoplasmic antibody-associated vasculitis; Ear, nose, and throat manifestations; Hypertrophic pachymeningitis
    • Accession Number:
      0 (Antibodies, Antineutrophil Cytoplasmic)
      0 (Immunosuppressive Agents)
      8N3DW7272P (Cyclophosphamide)
      EC 1.11.1.7 (Peroxidase)
    • Publication Date:
      Date Created: 20230416 Date Completed: 20230530 Latest Revision: 20230530
    • Publication Date:
      20240829
    • Accession Number:
      10.1016/j.autrev.2023.103338
    • Accession Number:
      37062439