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http://dx.doi.org/10.4046/trd.2009.67.6.569

A Case of Idiopathic Pulmonary Alveolar Proteinosis Treated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) after Partial Response to Whole Lung Lavage  

Song, Jun Whi (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Masan Samsung Hospital, Sungkyunkwan University School of Medicine)
Park, Sun Hyo (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Masan Samsung Hospital, Sungkyunkwan University School of Medicine)
Kang, Kyung Woo (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Masan Samsung Hospital, Sungkyunkwan University School of Medicine)
Publication Information
Tuberculosis and Respiratory Diseases / v.67, no.6, 2009 , pp. 569-573 More about this Journal
Abstract
Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by surfactant component accumulation in the alveolar space. Idiopathic PAP has recently been recognized as a autoimmune disease of impaired alveolar macrophage function caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). While whole lung lavage has been the standard treatment, not every patient shows a complete response. Subcutaneous injection or inhalation of GM-CSF is another promising treatment option for PAP. A 45-year-old patient visited our hospital for dyspnea, he was diagnosed as PAP and underwent whole lung lavage. Eighteen months later, the patient had not achieved complete remission in despite of initial response. After then he was administered with GM-CSF (5 ${mu}g/kg/day$, subcutaneous injection) for fivetimes a week during 2 months. Nine months later, the abnormal shadows in high-resolution computed tomography (HRCT) decreased and the patient fully recovered in forced vital capacity. After 60 months, the HRCT scan showed complete remission of PAP.
Keywords
Pulmonary Alveolar Proteinosis; Granulocyte-Macrophage Colony-Stimulating Factor, Bronchoalveolar Lavage;
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