耳症状で発症し急速に増悪した全身型ウェゲナー肉芽腫症の1例

• 首藤 純

  大分大学医学部免疫アレルギー統御講座 (耳鼻咽喉科)

• 末永 智

  大分大学医学部免疫アレルギー統御講座 (耳鼻咽喉科)

• 立山 香織

  大分大学医学部免疫アレルギー統御講座 (耳鼻咽喉科)

• 織部 加奈子

  大分大学医学部免疫アレルギー統御講座 (耳鼻咽喉科)

• 鈴木 正志

  大分大学医学部免疫アレルギー統御講座 (耳鼻咽喉科)

書誌事項

タイトル別名

• Acute Onset Wegener's Granulomatosis Presenting as Otitis Media ; A Case Report
• ジショウジョウ デ ハッショウシ キュウソク ニ ゾウアクシタ ゼンシンガタ ウェゲナー ニクガシュショウ ノ 1レイ

抄録

We report the case of a 57-year-old woman with Wegener's granulomatosis who presented with otitis media. The patient presented with a 2-month history of bilateral hearing loss and dizziness. Antibiotic treatment was not effective, and the patient was confirmed to have bilateral sensorineural hearing loss. Serum was positive for cytoplasmic anti-neutrophil cytoplasmic antibody [C(PR3)-ANCA] but negative for P(MPO)-ANCA (perinuclear ANCA). While the test results were pending, the patients' general condition worsened ; in particular, signs of active inflammation, cardiorespiratory failure and scleritis developed suddenly. Emergency chest computed tomography revealed evidence of lung involvement and hydrothorax ; thus, the patient was diagnosed to have the generalized form of Wegener's granulomatosis. The patient was started on pulse methylprednisolone therapy at 1000mg/day for 3 days, which resulted in marked clinical improvement, and then, the drug therapy was switched to prednisolone 60mg/day and cyclophosphamide 50mg/day and gradually tapered.<br>Early diagnosis of Wegener's granulomatosis is often difficult because of atypical manifestations of the disease ; particular attention must be paid to acute onset of the disease, such as in our case. ANCA is a very useful marker for early diagnosis, but about one week is needed to obtain the test results. We believe that early steroid and cyclophosphamide therapy is an effective therapeutic option for patients with signs of severe inflammation and generalized involvement.

収録刊行物

• 日本耳鼻咽喉科学会会報

  日本耳鼻咽喉科学会会報 110 (3), 107-110, 2007

  一般社団法人 日本耳鼻咽喉科頭頸部外科学会

キーワード

• ウェゲナー肉芽腫
• ステロイド・サイクロフォスファミド療法
• 難治性中耳炎
• ANCA

詳細情報

• CRID: 1390001205005042688
• NII論文ID: 10019478075
• NII書誌ID: AN00191551
• DOI: 10.3950/jibiinkoka.110.107
• COI: 1:STN:280:DC%2BD2s3hsFertw%3D%3D
• ISSN: 18830854
• ISSN: 00306622
• NDL書誌ID: 8758246
• PubMed: 17419446
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I8758246
• Web Site: https://search.jamas.or.jp/link/ui/2007154122
• データソース種別:
  • JaLC
  • NDL
  • Crossref
  • PubMed
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可