Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment

스테로이드 치료로 호전을 보인 폐동맥 고혈압을 유발한 특발성 섬유화성 종격동염 1예

  • Jung, Bo Yong (Department of Internal Medicine, University of Soonchunhyang College of Medicine) ;
  • Eo, Sung Jun (Department of Internal Medicine, University of Soonchunhyang College of Medicine) ;
  • Park, Eun Seo (Department of Internal Medicine, University of Soonchunhyang College of Medicine) ;
  • Kim, Young Tong (Department of Radiology, University of Soonchunhyang College of Medicine) ;
  • Cho, Jae Sung (Department of Internal Medicine, University of Soonchunhyang College of Medicine) ;
  • Oh, Mi Hye (Department of Diagnostic Pathology, University of Soonchunhyang College of Medicine) ;
  • Seo, Gi Hyun (Department of Internal Medicine, University of Soonchunhyang College of Medicine) ;
  • Na, Joo Ock (Department of Internal Medicine, University of Soonchunhyang College of Medicine) ;
  • Lee, Sang Do (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Uh, Soo Taek (Department of Internal Medicine, University of Soonchunhyang College of Medicine) ;
  • Kim, Yong Hoon (Department of Internal Medicine, University of Soonchunhyang College of Medicine) ;
  • Park, Choon Sik (Department of Internal Medicine, University of Soonchunhyang College of Medicine)
  • 정보용 (순천향대학교 의과대학 내과학교실) ;
  • 어성준 (순천향대학교 의과대학 내과학교실) ;
  • 박은서 (순천향대학교 의과대학 내과학교실) ;
  • 김영통 (순천향대학교 의과대학 영상의학교실) ;
  • 최재성 (순천향대학교 의과대학 내과학교실) ;
  • 오미혜 (순천향대학교 의과대학 진단병리교실) ;
  • 서기현 (순천향대학교 의과대학 내과학교실) ;
  • 나주옥 (순천향대학교 의과대학 내과학교실) ;
  • 이상도 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 어수택 (순천향대학교 의과대학 내과학교실) ;
  • 김용훈 (순천향대학교 의과대학 내과학교실) ;
  • 박춘식 (순천향대학교 의과대학 내과학교실)
  • Received : 2006.04.18
  • Accepted : 2006.06.16
  • Published : 2006.07.30

Abstract

Idiopathic fibrosing mediastinitis is, an uncommon cause of pulmonary hypertension this is characterized by excessive fibrosis of the mediastinum with an unknown etiology. Steroid therapy has been suggested for individuals with progressive symptoms, bu there is littlet data demonstrating the efficacy of such therapy are lacking. We present a case of pulmonary hypertension secondary to a compression of a main pulmonary artery by fibrosing mediastinitis which was confirmed by a biopsy with a thoracotomy. The chest CT scan and 2D echocardiography performed before and after a trial of steroid therapy demonstrated improvement after steroid therapy.

저자들은 종격동에 전반적인 섬유화성 연부조직 침윤으로 인해 폐동맥 협착을 일으켜 호흡곤란과 폐동맥 고혈압이 발생한 특발성 섬유화성 종격동염을 진단하고, 경구 부신피질 호르몬 투여 후 호전을 보인 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Keywords

References

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