全肺灌洗配合巨噬细胞集落刺激因子治疗肺泡蛋白沉着症1例

Treamtment of pulmonary alveolar proteinosis with whole lung lavage and granulocyte macrophage colony stimulating factor in 1 case

肺泡蛋白沉着症(PAP)是一种临床少见的间质性肺疾病,由于支气管或肺泡表面大量富磷脂蛋白样物质沉积所致,常出现渐进性呼吸困难,伴干咳无痰或少量白色黏痰、乏力、体重减轻、低热等症状,可通过过碘酸雪夫染色及经胸腔镜或支气管肺活检获取肺组织以明确诊断。全肺肺泡灌洗被认为是有效且安全的治疗方法,而近年来,重组人粒细胞巨噬细胞集落刺激因子替代治疗的使用,更有利于PAP的治疗。

Pulmonary alveolar proteinosis（PAP） is a rare clinical interstitial lung disease characterized by the build-up of lipoprotein material on the surface of bronchal or alveoli.The disease often causes progressive dyspnea,dry cough without sputum or with a small amount of white sticky phlegm,fatigue,weight loss,low-grade fever and other symptoms. The diagnosis can be confirmed through periodic acid-Schiff stain and transbronchial lung biopsy.Whole lung lavages is considered as the most effective and safe.In recent years,the use of granulocyte macrophage colony stimulating factor is more conducive to the treatment of PAP.