Rodríguez Portal José Antonio, Rodríguez Becerra Eulogio, Sánchez Garrido Antonio
Unidad Médico-Quirúrgica de Enfermedades Respiratorias, Hospitales Universitarios Virgen del Rocío, Sevilla, España.
Arch Bronconeumol. 2009 Mar;45(3):150-2. doi: 10.1016/j.arbres.2008.02.006. Epub 2009 Feb 10.
Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinaceous material derived from alveolar surfactant in the alveoli, with a consequent deterioration in gas exchange. Pathogenesis is related to impaired phagocytic function of alveolar macrophages. In recent years, a new treatment for pulmonary alveolar proteinosis-consisting of subcutaneous administration of granulocyte-macrophage colony-stimulating factor (GM-CSF)-has become available. The commonly accepted treatment, and the one to have shown greatest efficacy in pulmonary alveolar proteinosis, is whole lung lavage. Instead of subcutaneous administration, GM-CSF can also be inhaled as an aerosol. This route of administration of GM-CSF is safe and effective in the treatment of pulmonary alveolar proteinosis and represents an alternative to subcutaneous administration or whole lung lavage. We present a patient with pulmonary alveolar proteinosis who was treated with inhaled GM-CSF and describe her clinical and functional outcome after 1 year of treatment.
肺泡蛋白沉积症是一种罕见疾病，其特征是肺泡内出现源自肺泡表面活性物质的脂蛋白样物质积聚，进而导致气体交换功能恶化。发病机制与肺泡巨噬细胞吞噬功能受损有关。近年来，一种治疗肺泡蛋白沉积症的新方法——皮下注射粒细胞巨噬细胞集落刺激因子（GM-CSF）——已可供使用。目前公认的治疗方法，也是在肺泡蛋白沉积症中显示出最大疗效的方法，是全肺灌洗。GM-CSF除了皮下注射外，也可以以气雾剂形式吸入。GM-CSF的这种给药途径在治疗肺泡蛋白沉积症方面安全有效，是皮下注射或全肺灌洗的一种替代方法。我们报告一名接受吸入GM-CSF治疗的肺泡蛋白沉积症患者，并描述其治疗1年后的临床和功能结果。
