A Case of Idiopathic Pulmonary Alveolar Proteinosis Treated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) after Partial Response to Whole Lung Lavage.
10.4046/trd.2009.67.6.569
- Author:
Jun Whi SONG
1
;
Sun Hyo PARK
;
Kyung Woo KANG
Author Information
1. Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Masan Samsung Hospital, Sungkyunkwan University School of Medicine, Masan, Korea. kangkw9@naver.com
- Publication Type:Case Report
- Keywords:
Pulmonary Alveolar Proteinosis;
Granulocyte-Macrophage Colony-Stimulating Factor, Bronchoalveolar Lavage
- MeSH:
Autoantibodies;
Autoimmune Diseases;
Bronchoalveolar Lavage;
Colony-Stimulating Factors;
Dyspnea;
Granulocyte-Macrophage Colony-Stimulating Factor;
Humans;
Inhalation;
Injections, Subcutaneous;
Lung;
Macrophages, Alveolar;
Middle Aged;
Pulmonary Alveolar Proteinosis;
Vital Capacity
- From:Tuberculosis and Respiratory Diseases
2009;67(6):569-573
- CountryRepublic of Korea
- Language:Korean
-
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 microgram/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.
