顔面神経麻痺と肥厚性硬膜炎を合併したMPO-ANCA陽性の難治性中耳炎の1例 A case of MPO-ANCA-positive refractory otitis media with facial nerve palsy and hypertrophic pachymeningitis

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著者

    • 大田 重人 OHTA Shigeto
    • 琉球大学医学部耳鼻咽喉・頭頸部外科 Department of Otorhinolaryngology, Head and Neck Surgery, Graduate School of Medicine, University of the Ryukyus
    • 我那覇 章 GANAHA Akira
    • 琉球大学医学部耳鼻咽喉・頭頸部外科 Department of Otorhinolaryngology, Head and Neck Surgery, Graduate School of Medicine, University of the Ryukyus
    • 鈴木 幹男 SUZUKI Mikio
    • 琉球大学医学部耳鼻咽喉・頭頸部外科 Department of Otorhinolaryngology, Head and Neck Surgery, Graduate School of Medicine, University of the Ryukyus

抄録

難治性中耳炎の経過中に、顔面神経麻痺と肥厚性硬膜炎を合併したMPO-ANCA陽性症例を経験した。症例は74歳女性で、右滲出性中耳炎にて鼓膜換気チューブ留置されたが、骨導閾値上昇したため当科紹介となった。細菌性内耳炎の診断で、抗生剤とステロイド点滴を行った。血液検査ではPR3-ANCA陰性、MPO-ANCA陽性であった。ステロイド内服継続して通院中に、右顔面神経麻痺が出現し、ステロイドミニパルスを行った。乳突削開術での病理検査は、非特異的炎症性肉芽であった。退院して、ステロイド維持内服していたが、多発神経症状が出現し、MRIで肥厚性硬膜炎を認めた。ステロイドパルスと免疫抑制剤投与を開始し、症状は改善した。MPO-ANCAのみ陽性で、病理組織学的検査でも特異的所見が得られない場合は、確定診断に苦慮する。Wegener肉芽腫症を含めたANCA関連血管炎症候群として早期に治療を開始することが必要と考えた。

A 74-year-old woman complained of right aural fullness. She was diagnosed as secretory otitis media in her right ear and received a transtympanic ventilation tube insertion accompanied with antibiotic treatment. However, ear discharge through ventilation tube had been persisting and her symptoms were deteriorated. She was referred to our hospital because of mixed conductive-sensorineural hearing loss and dizziness. We suspected of bacterial labyrinthitis and treated with antibiotics and steroids (hydrocortisone). Although ear discharge and dizziness were improved after treatment, she had right facial nerve palsy and sever headache. Gadolinium-enhanced MRI revealed a thickening of dura mater in the right middle cranial fossa. Serum concentration of myeloperoxidase-antineutrophil cytoplasmic autoantibody (MPO-ANCA) was elevated. But proteinase 3 (PR3)-ANCA was negative. Histopathological examination of the specimen taken by right exploratory tympanomastoidectomy revealed non-specific inflammatory granulation tissue. Pulmonary and renal involvements were not detected. Thus, we diagnosed her disease as hypertrophic pachymeningitis associated with ANCA-associated vasculitis including a localized type of Wegener's granulomatosis (WG). After the administration of methylprednisolone and cyclophosphamide, her symptoms were sufficiently improved and the serum level of MPO-ANCA became normalized.<br> The treatment experience demonstrated that we have to make an early diagnosis of ANCA- associated vasculitis including WG and started steroids therapy along with immunosuppressant, even if the results of histopathological examination were not specific and MPO-ANCA was only positive.

収録刊行物

  • Otology Japan

    Otology Japan 21(5), 800-807, 2011-12-26

    THE JAPAN OTOLOGICAL SOCIETY

参考文献:  23件中 1-23件 を表示

被引用文献:  3件中 1-3件 を表示

各種コード

  • NII論文ID(NAID)
    10030285287
  • NII書誌ID(NCID)
    AN10358085
  • 本文言語コード
    JPN
  • 資料種別
    ART
  • ISSN
    09172025
  • データ提供元
    CJP書誌  CJP引用  J-STAGE 
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