Diffuse Reticular Interstitial Infiltrations in the Patient with Worsening Exertional Dyspnea after Clomifen Hormonal Therapy

Clomifen 호르몬제 복용후 호흡곤란이 악화된 환자에서의 미만성 망상형 간질성 폐침윤

  • Park, Hyeong-Cheon (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kim, Young-Sam (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kim, Se-Kyu (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Chung, Kyung-Young (Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine) ;
  • Shin, Dong-Hwan (Department of Pathology, Yonsei University College of Medicine) ;
  • Lee, Hong-Lyeol (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Chang, Joon (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Kim, Sung-Kyu (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Lee, Won-Young (Department of Internal Medicine, Yonsei University College of Medicine)
  • 박형천 (연세대학교 의과대학 내과학교실 폐질환연구소) ;
  • 김영삼 (연세대학교 의과대학 내과학교실 폐질환연구소) ;
  • 김세규 (연세대학교 의과대학 내과학교실 폐질환연구소) ;
  • 정경영 (연세대학교 의과대학 흉부외과학교실 폐질환연구소) ;
  • 신동환 (연세대학교 의과대학 병리학교실 폐질환연구소) ;
  • 이홍렬 (연세대학교 의과대학 내과학교실 폐질환연구소) ;
  • 장준 (연세대학교 의과대학 내과학교실 폐질환연구소) ;
  • 김성규 (연세대학교 의과대학 내과학교실 폐질환연구소) ;
  • 이원영 (연세대학교 의과대학 내과학교실 폐질환연구소)
  • Published : 1995.08.31

Abstract

Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. We experienced a 32-year-old female who was admitted because of worsening exertional dyspnea after hormonal therapy with Clomifen for five months and intermittent hemoptysis. Chest PA showed diffuse ground glass appearance with some reticular infiltrations. High resolution CT scan showed multiple small thin walled cysts distributed homogeneously throughout the entire lung. Pulmonary function test showed characteristic obstructive pattern despite the restrictive interstitial pattern of chest radiography. Thoracoscopic lung biopsy specimen showed abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and pulmonary vessels typical of pulmonary lymphangioleiomyomatosis. Hormonal therapy with medroxyprogesterone was initiated.

Keywords