近年在肺泡蛋白沉着症的发病机制和治疗研究方面取得了一定的进展 ,肺泡表面活性物质代谢的异常和肺泡巨噬细胞清除功能的下降导致肺泡腔内大量表面活性蛋白和脂质的沉积。动物实验模型显示粒细胞 巨噬细胞集落刺激因子 (GM CSF)的缺乏或其受体不足与本病的发生有关。应用GM CSF治疗有可能取代传统的治疗方法。 In recent years, some progress has been made in the pathogenesis and treatment of pulmonary alveolar proteinosis. Abnormalities of alveolar surfactant metabolism and the clearance of alveolar macrophages result in deposition of a large number of surface active proteins and lipids in the alveolar space. Animal experimental models show that the lack of granulocyte macrophage colony stimulating factor (GM CSF) or its receptor deficiency and the occurrence of this disease. GM CSF therapy may replace the traditional treatment.