Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
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Diffuse Reticular Interstitial infiltrations Accompanied by Hyperinflation

과팽창이 동반된 미만성 망상형 간질성 폐침윤

  • Lee, Kye-Young (Department of Internal Medicine, Seoul National University College of Medicine) ;
  • Kim, Young-Whan (Department of Internal Medicine, Seoul National University College of Medicine) ;
  • Han, Sung-Koo (Department of Internal Medicine, Seoul National University College of Medicine) ;
  • Shim, Young-Soo (Department of Internal Medicine, Seoul National University College of Medicine) ;
  • Kim, Keun-Youl (Department of Internal Medicine, Seoul National University College of Medicine) ;
  • Han, Yong-Chol (Department of Internal Medicine, Seoul National University College of Medicine)
  • 이계영 (서울대학교 의과대학 내과학교실) ;
  • 김영환 (서울대학교 의과대학 내과학교실) ;
  • 한성구 (서울대학교 의과대학 내과학교실) ;
  • 심영수 (서울대학교 의과대학 내과학교실) ;
  • 김건열 (서울대학교 의과대학 내과학교실) ;
  • 한용철 (서울대학교 의과대학 내과학교실)
  • Published : 1993.02.28
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Abstract

A 38-year-old female was admitted due to progressive exertional dyspnea and intermittent blood tinged sputum. Chest PA showed diffuse reticular infiltration accompanied by hyperinflation. $PaO_2$ was normal in resting state but profoundly decreased during exercise. Spirometry showed severe obstructive pattern but DLCO was markedly decreased. Lung volumes measured by helium equilibrium method was increased. On HRCT, numerous and relatively uniform sized cysts were evenly distributed throughout the bilateral lung fields. Open lung biopsy was performed and pulmonary lymphangioleiomyomatosis(LAM) was diagnosed based on the findings of abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and small pulmonary veins. Immunohistochemical staining was negative for estrogen receptor but positive for progesterone receptor. Medroxyprogesterone therapy was initiated.

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