Seven cases illustrating difficulties in the treatment of MPO-ANCA-positive refractory otitis media.

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  • Author(s): Otsuki K;Otsuki K; Imaizumi M; Imaizumi M; Murono S; Murono S
  • Source:
    Fukushima journal of medical science [Fukushima J Med Sci] 2024 Oct 18; Vol. 70 (4), pp. 175-182. Date of Electronic Publication: 2024 Oct 04.
  • Publication Type:
    Journal Article; Case Reports
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: Fukushima Society of Medical Science Country of Publication: Japan NLM ID: 0374626 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2185-4610 (Electronic) Linking ISSN: 00162590 NLM ISO Abbreviation: Fukushima J Med Sci Subsets: MEDLINE
    • Publication Information:
      Publication: Fukushima : Fukushima Society of Medical Science
      Original Publication: Fukushima, Japan. Fukushima Kenritsu Ika Daigaku.
    • Subject Terms:
      Otitis Media*/drug therapy ; Peroxidase*/immunology ; Antibodies, Antineutrophil Cytoplasmic*/blood ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis*/drug therapy; Humans ; Female ; Male ; Middle Aged ; Aged ; Aged, 80 and over
    • Abstract:
      There are increasing reports of patients with refractory otitis media caused by anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), especially myeloperoxidase (MPO)-ANCA-positive middle ear disease.　However, making a definitive diagnosis can be difficult, which can adversely affect the outcome of treatment.　We reviewed the diagnostic features of MPO-ANCA-positive middle ear disease and here discuss the difficulties of timely diagnosis and treatment.　Seven cases were eligible (6 women, 1 man;aged 57-83 years), and all were MPO-ANCA positive and proteinase 3 (PR3)-ANCA negative.　The patients were referred to our institution for management of intractable otitis media (2/7), progressive hearing loss (7/7) with facial palsy (1/7), and/or a high MPO-ANCA titer (5/7).　All patients underwent tapering steroid therapy and their MPO-ANCA titer was monitored.　Refractory MPO-ANCA-positive otitis media was noted:5 of 7 cases showed improvement with tapering steroid therapy but cure was not achieved in the remaining 2 cases.　This study demonstrates the difficulties in the diagnosis and treatment of localized AAV.　Early diagnosis and treatment can improve the prognosis of patients with AAV but global diagnostic criteria for ear disease have not been established.　Additional cases should be prospectively examined to establish a treatment for MPO-ANCA-positive middle ear disease.
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    • Contributed Indexing:
      Keywords: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV); myeloperoxidase (MPO)-ANCA; proteinase 3 (PR3)-ANCA
    • Accession Number:
      EC 1.11.1.7 (Peroxidase)
      0 (Antibodies, Antineutrophil Cytoplasmic)
    • Publication Date:
      Date Created: 20241006 Date Completed: 20241020 Latest Revision: 20241211
    • Publication Date:
      20241211
    • Accession Number:
      PMC11625855
    • Accession Number:
      10.5387/fms.2023-21
    • Accession Number:
      39370277