A Case of Pulmonary Alveolar Proteinosis

폐포단백증 1예

  • Woo, Dae-Hyung (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Park, Jung-Eun (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Ryu, Yung-Ha (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Kim, Hyun-Jung (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Shin, Kyeong-Cheol (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Chung, Jin-Hong (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Lee, Kwan-Ho (Department of Internal Medicine, College of Medicine, Yeungnam University)
  • 우대형 (영남대학교 의과대학 내과학교실) ;
  • 박정은 (영남대학교 의과대학 내과학교실) ;
  • 류영하 (영남대학교 의과대학 내과학교실) ;
  • 김현정 (영남대학교 의과대학 내과학교실) ;
  • 신경철 (영남대학교 의과대학 내과학교실) ;
  • 정진홍 (영남대학교 의과대학 내과학교실) ;
  • 이관호 (영남대학교 의과대학 내과학교실)
  • Published : 2010.06.30

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare disorder that's characterized by accumulation of surfactant components in the alveolar space. Idiopathic PAP is recognized as an autoimmune disease that's due to impaired alveolar macrophage function and this caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). We report here a case of pulmonary alveolar proteinosis that was deemed interstitial lung disease at the initial diagnosis. A 61-year-old man presented with intermittent blood tinged sputum and dyspnea on exertion. The man was a painter for 30 years and he had a 10 pack-years smoking history. Chest computerized tomography (CT) revealed multifocal ground-glass opacity with interstitial thickening at both lungs. His pulmonary function tests and methacholine test revealed non specific results. He was diagnosed with interstitial lung disease on the basis of the chest CT finding and occupational history. However, seven months later, his symptoms progressed. Follow-up chest CT was performed. Wedge resection via video-assisted thoracoscopic surgery (the anterior basal segment of the left lower lobe) was done. Microscopic examination showed large groups of alveoli with excessive amounts of surfactant and a complex mixture of protein and lipid (fat) molecules. Finally, he was diagnosed as having pulmonary alveolar proteinosis.

Keywords