A Case of Wegener's Granulomatosis with Delayed Diagnosis due to Clinical Features of Henoch-Schönlein Purpura

Henoch-Schölein 자반증 임상양상으로 나타나 진단이 늦어졌던 웨게너 육아종증 1예

  • Song, Se Bin (Department of Pulmonary and Critical Care Medicine, Kyunghee University College of Medicine) ;
  • Choi, Hye Sook (Department of Pulmonary and Critical Care Medicine, Kyunghee University College of Medicine) ;
  • Kim, Yee Hyung (Department of Pulmonary and Critical Care Medicine, Kyunghee University College of Medicine) ;
  • Choi, Cheon Woong (Department of Pulmonary and Critical Care Medicine, Kyunghee University College of Medicine) ;
  • Park, Myung Jae (Department of Pulmonary and Critical Care Medicine, Kyunghee University College of Medicine) ;
  • Yoo, Jee-Hong (Department of Pulmonary and Critical Care Medicine, Kyunghee University College of Medicine) ;
  • Kang, Hong Mo (Department of Pulmonary and Critical Care Medicine, Kyunghee University College of Medicine) ;
  • Kim, Yoon Hwa (Department of Pathology, Kyunghee University College of Medicine) ;
  • Park, Joo Cheol (Department of Thoracic Surgery, Kyunghee University College of Medicine)
  • 송세빈 (경희대학교 의과대학 호흡기내과학교실) ;
  • 최혜숙 (경희대학교 의과대학 호흡기내과학교실) ;
  • 김이형 (경희대학교 의과대학 호흡기내과학교실) ;
  • 최천웅 (경희대학교 의과대학 호흡기내과학교실) ;
  • 박명재 (경희대학교 의과대학 호흡기내과학교실) ;
  • 유지홍 (경희대학교 의과대학 호흡기내과학교실) ;
  • 강홍모 (경희대학교 의과대학 호흡기내과학교실) ;
  • 김윤화 (경희대학교 의과대학 병리학교실) ;
  • 박주철 (경희대학교 의과대학 흉부외과학교실)
  • Received : 2007.10.19
  • Accepted : 2007.12.03
  • Published : 2007.12.30

Abstract

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-$Sch\ddot{o}nlein$ purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-$Sch\ddot{o}nlein$ purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.

웨게너 육아종증은 중형내지 소형의 동맥과 정맥을 침범하는 전신적 혈관염의 한 형태로 상, 하부 기도 및 신장의 질환을 특징으로 한다. 신장에 국한된 질환으로 나타날 때에는 Henoch-$Sch\ddot{o}nlein$ 자반증, 현미경적 다발성 혈관염 등의 기타 신장 침범 혈관염과 구분하기 어렵다. 본 증례에서는 초기 신조직검사에서 IgA 양성으로 Henoch-$Sch\ddot{o}nlein$ 자반증으로 진단되고, 15년 후에 폐조직 검사에서 웨게너 육아종증으로 진단된 1예를 경험하였기에 문헌고찰과 함께 보고한다.

Keywords

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