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Published in: European Archives of Oto-Rhino-Laryngology 12/2018

Open Access 01-12-2018 | Review Article

Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature

Authors: Anquan Peng, Xinming Yang, Weijing Wu, Zian Xiao, Dinghua Xie, Shenglei Ge

Published in: European Archives of Oto-Rhino-Laryngology | Issue 12/2018

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Abstract

Background and objective

It has been recognized that anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides may lead to hypertrophic pachymeningitis (HP) or intractable otitis media (OM). To our knowledge, few cases of coexistent ANCA-related HP and OM have been described previously. To increase awareness of this disease, we reviewed the literature describing patients with HP and intractable OM in a population with AAV to guide clinical decision making for otolaryngologists.

Methods

 PubMed was searched with the following terms: ANCA-associated vasculitis, otitis media, and hypertrophic pachymeningitis. Only patients with concomitant AAV, OM and HP were considered and included in this review.

Results

A total of 243 articles were reviewed, and of these, 6 met inclusion criteria. Headache, cranial polyneuropathy, and intractable OM with effusion or granulation were common. Serum MPO–ANCA positivity was most common in Asian patients. Almost all patients had dural mater thickening on gadolinium-enhanced magnetic resonance imaging of the brain. Corticosteroids plus an immunosuppressant was more effective and most patients had improved hearing after treatment, but approximately 50% of subjects had disease relapse.

Conclusion

In this review, we summarized the current knowledge on the clinical features, diagnosis, treatment, and pathogenesis of this disease. We should carefully detect the potential cases of ANCA-related HP and OM in patients with intractable OM, HP, or AAV, and make the optimal treatment plan to avoid long-term neurological complications and irreversible hearing loss. Furthermore, due to an increased possibility of relapse, close follow-up, including a hearing test, ANCA titers, imaging examination, and detection of toxic and side effects of immunosuppressive therapy, are necessary.
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Metadata
Title
Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature
Authors
Anquan Peng
Xinming Yang
Weijing Wu
Zian Xiao
Dinghua Xie
Shenglei Ge
Publication date
01-12-2018
Publisher
Springer Berlin Heidelberg
Published in
European Archives of Oto-Rhino-Laryngology / Issue 12/2018
Print ISSN: 0937-4477
Electronic ISSN: 1434-4726
DOI
https://doi.org/10.1007/s00405-018-5172-4

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