Korean J Med.
2012 Mar;82(3):357-361.
A Case of Pulmonary Alveolar Proteinosis Improved with Inhaled Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)
- Affiliations
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- 1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
- 2Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. skysong3@hanmail.net
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.
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