A Case of Churg-Strauss Syndrome with Multiple Tracheobronchial Mucosal Lesions

기관과 기관지내 다발성 점막 병변을 동반한 Churg-Strauss 증후군 1예

  • Boo, Sun-Jin (Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Lee, Kwangha (Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Ra, Seung Won (Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Jin, Young-Joo (Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Park, Gyung-Min (Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Hong, Sang-Bum (Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center)
  • 부선진 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 이광하 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 나승원 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 진영주 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 박경민 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 홍상범 (울산대학교 의과대학 서울아산병원 호흡기내과)
  • Received : 2008.07.18
  • Accepted : 2008.08.19
  • Published : 2008.11.30

Abstract

Churg-Strauss syndrome is a rare form of systemic necrotizing vasculitis that occurs exclusively in patients with asthma, and is associated with blood and tissue eosinophilia. The classic pathology findings in the lung include a combination of eosinophilic pneumonia, granulomatous inflammation and necrotizing vasculitis. However, there are few reports of tracheobronchial mucosal lesions in Churg-Strauss syndrome. We report a case of Churg-Strauss syndrome with multiple tracheobronchial mucosal lesions in a 33-year-old man with a history of bronchial asthma and allergic rhinitis. He had been diagnosed with community acquired pneumonia at another hospital and was treated with antibiotics. However, the chest radiographic findings were aggravated and showed multifocal consolidations in the whole lung fields. He was transferred to the Asan Medical Center. Fiberoptic bronchoscopy revealed multiple nodular mucosal lesions of the trachea and bronchi. The histopathology of the mucosal lesions revealed necrotizing bronchial inflammation with eosinophilic infiltration. Video Assisted Thoracic Surgery was performed. The wedge resected lung tissue revealed chronic eosinophilic pneumonia that was consistent with Churg-Strauss syndrome. Methylprednisolone (1 mg/kg q 8 hr) was prescribed and his symptoms resolved gradually. The chest radiographic findings improved significantly, and a follow-up fiberoptic bronchoscopy performed eight days later showed that the tracheobronchial mucosal lesions had resolved. The patient was prescribed oral prednisolone for 20 months after discharge. Currently, the patient is not taking steroids and is being followed up.

본 예는 Churg-Strauss 증후군에서의 폐침윤은 주로 폐실질에 호산구성 폐렴, 괴사성 혈관염 및 육아종성 염증의 형태로 발현이 되나 기관 기관지 내 점막 병변으로도 발현될 수 있음을 보여 주었다. 저자들은 기관지 천식과 반복되는 비염의 병력을 가진 33세 남자가 호흡곤란을 주소로 내원하여 Churg-Strauss 증후군으로 진단되었고 동반된 기관지 내시경상 기관과 기관지내 점막 병변이 스테로이드로 치료하면서 호전된 1예를 경험하였기에 보고하는 바이다.

Keywords

References

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