The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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A Case of Pulmonary Alveolar Proteinosis Improved with Inhaled Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)

GM-CSF 흡입치료로 호전된 폐포단백증 1예

  • Kang, Bo-Hyoung (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Ho-Su (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Yu-Mi (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park, So-Eun (Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Woo-Sung (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Dong-Soon (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Song, Jin-Woo (Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine)
  • 강보형 (울산대학교 의과대학 서울아산병원 내과) ;
  • 이호수 (울산대학교 의과대학 서울아산병원 내과) ;
  • 이유미 (울산대학교 의과대학 서울아산병원 내과) ;
  • 박소은 (울산대학교 의과대학 서울아산병원 내과) ;
  • 김우성 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 김동순 (울산대학교 의과대학 서울아산병원 호흡기내과) ;
  • 송진우 (울산대학교 의과대학 서울아산병원 호흡기내과)
  • Published : 2012.03.01
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Abstract

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of lipoproteinaceous material within the alveoli. Several studies have recently found that autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) play a major role in the pathogenesis of idiopathic PAP. Consequently, inhaled or systemic injection of GM-CSF has been suggested as a promising treatment for PAP. A 54-year-old male visited our hospital for progressive dyspnea. Four years earlier, he was diagnosed with PAP based on a surgical lung biopsy in another institution. Whole-lung lavage was performed four times before he visited our hospital. We administered high-dose inhaled GM-CSF therapy for 12 weeks followed by 12 weeks of low-dose therapy. After the GM-CSF treatment, the patient's symptoms, lung function, and radiological findings were improved significantly.

폐포단백증은 폐포에 표면활성제 기원의 인지질이 비정상적으로 다량 침착되는 드문 질병으로 자연 관해에서 호흡부전으로 인한 사망까지 다양한 임상경과를 보인다. 폐포단백증의 치료로는 현재까지 전폐 세척술이 표준치료로 받아 들여지고 있다. 하지만 전신마취 및 기관삽관 등이 필요한 침습적 시술이라는 위험성이 있어 최근에는 GM-CSF를 이용한 치료법들이 연구되고 있다. 본 증례는 내원 4년 전 폐포단백증으로 진단받은 환자로 호흡곤란 및 방사선학적 소견의 악화로 전폐 세척술을 4차례 시행 받았으나 잦은 재발로 GM-CSF 흡입치료를 시작한 환자로 흡입치료 후 증상의 호전 및 방사선학적 소견을 경험한 예이다.

Keywords

References

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