title: Clinical features and treatment outcomes of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV): A retrospective analysis of 235 patients from a nationwide survey in Japan.
abstract: OBJECTIVE
  We aimed to analyze clinical features and treatment outcomes of otitis media caused by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), i.e. otitis media with AAV (OMAAV).
  
  
  METHODS
  This survey was performed between December 2013 and February 2014. The study began with a preliminary survey to 123 otolaryngology institutions in Japan to inquire about their experiences with OMAAV patients during the past 10 years, and was followed by a questionnaire survey to investigate clinical and laboratory findings. OMAAV was defined using the criteria described in the text.
  
  
  RESULTS
  Two hundred and thirty-five patients classified as OMAAV were enrolled in this study. They were characterized as follows: (1) disease onset with initial signs/symptoms due to intractable otitis media with effusion or granulation, which did not respond to ordinary treatments such as antibiotics and insertion of tympanic ventilation tubes, followed by progressive hearing loss; (2) predominantly female (73%) and older (median age: 68 years); (3) predominantly myeloperoxidase (MPO)-ANCA-positive (60%), followed by proteinase 3 (PR3)-ANCA-positive (19%) and both ANCAs-negative (16%); (4) frequently observed accompanying facial palsy (36%) and hypertrophic pachymeningitis (28%); and (5) disease often involving lung (35%) and kidney (26%) lesions. Four factors associated with OMAAV were found to be related to an unfavorable clinical course threatening the patient's hearing and/or lives, namely facial palsy, hypertrophic pachymeningitis, both ANCAs-negative phenotype, and disease relapse. The occurrence of hypertrophic pachymeningitis was associated with facial palsy (p < 0.05), both ANCAs-negative phenotype (p < 0.001), and headache (p < 0.001). The administration of corticosteroid together with an immunosuppressant was an independent predicting factor for lack of disease relapse (odds ratio [OR] = 1.90, p = 0.03) and an improvement in hearing loss (OR =2.58, p = 0.0002).
  
  
  CONCLUSIONS
  Since OMAAV has novel clinical features, the disease may be categorized as a subentity for the classification of AAV.
authors.affiliation: a Department of Otolaryngology-Head and Neck Surgery , Asahikawa Medical University , Asahikawa , Hokkaido , Japan.
authors.forename: Yasuaki
authors.initials: Y
authors.lastname: Harabuchi
authors.affiliation: a Department of Otolaryngology-Head and Neck Surgery , Asahikawa Medical University , Asahikawa , Hokkaido , Japan.
authors.forename: Kan
authors.initials: K
authors.lastname: Kishibe
authors.affiliation: b Department of Otolaryngology , Oita University Faculty of Medicine , Oita , Japan.
authors.forename: Kaori
authors.initials: K
authors.lastname: Tateyama
authors.affiliation: c Department of Otolaryngology , Niigata University Faculty of Medicine , Niigata , Japan.
authors.forename: Yuka
authors.initials: Y
authors.lastname: Morita
authors.affiliation: d Department of Otolaryngology , Jichi Medical University Saitama Medical Center , Omiya , Japan.
authors.forename: Naohiro
authors.initials: N
authors.lastname: Yoshida
authors.affiliation: e Department of Otolaryngology-Head and Neck Surgery , Tottori University Faculty of Medicine , Tottori , Japan.
authors.forename: Yasuomi
authors.initials: Y
authors.lastname: Kunimoto
authors.affiliation: f Department of Otolaryngology , Fukushima Medical University , Fukushima , Japan.
authors.forename: Takamichi
authors.initials: T
authors.lastname: Matsui
authors.affiliation: g Department of Otolaryngology-Head and Neck Surgery , Kyoto Prefectural University of Medicine , Kyoto , Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Sakaguchi
authors.affiliation: h Department of Otolaryngology , Ehime University Faculty of Medicine , Tonoo , Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Okada
authors.affiliation: i Department of Otolaryngology , Nagasaki University Faculty of Medicine , Nagasaki , Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Watanabe
authors.affiliation: j Department of Neuro-otolaryngology , Nagoya City University Graduate School of Medical Sciences , Nagoya , Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Inagaki
authors.affiliation: k Department of Internal Medicine , Juntendo University Koshigaya Hospital , Koshigaya , Japan , and.
authors.forename: Shigeto
authors.initials: S
authors.lastname: Kobayashi
authors.affiliation: d Department of Otolaryngology , Jichi Medical University Saitama Medical Center , Omiya , Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Iino
authors.affiliation: j Department of Neuro-otolaryngology , Nagoya City University Graduate School of Medical Sciences , Nagoya , Japan.
authors.forename: Shingo
authors.initials: S
authors.lastname: Murakami
authors.affiliation: i Department of Otolaryngology , Nagasaki University Faculty of Medicine , Nagasaki , Japan.
authors.forename: Haruo
authors.initials: H
authors.lastname: Takahashi
authors.affiliation: l Department of Otolaryngology , Miyazaki University Faculty of Medicine , Miyazaki , Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Tono
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000276:immunology / Q000628:therapy*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D001323:Autoantibodies; D005260:Female; D006306:Health Surveys; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology; D010033:Otitis Media / Q000209:etiology / Q000276:immunology / Q000628:therapy*; D009195:Peroxidase / Q000276:immunology; D012189:Retrospective Studies; D012737:Sex Factors; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D009195:Peroxidase; D053146:Myeloblastin
keywords: Antineutrophil cytoplasmic antibody; Eosinophilic granulomatosis with polyangiitis; Facial palsy; Granulomatosis with polyangiitis; Hypertrophic pachymeningitis; Microscopic polyangiitis; Myeloperoxidase-ANCA; Otitis media with ANCA-associated vasculitis; Proteinase 3 (PR3)-ANCA