关键词: 肺泡蛋白沉积症, 治疗, 全肺灌洗术

Abstract: Pulmonary alveolar proteinosis is a rare clinical syndrome, which was first reported by Rosen in 1958. It is characterized by the accumulation of phospholipidrich proteins(PAP)  that are positive forpositivperiodic acidSchiffstaining in the alveolar and bronchial lumens, leading to pulmonary ventilatory dysfunction. It was divided into three types: autoimmune PAP, congenital PAP and secondary PAP. Wholelung lavages is still the cornerstone of treatment, and GMCSF therapy, rituximab, plasma exchange and lung transplantation are emerging treatments. Most studies are in case series, often with limited patient numbers, so the level of evidence is low. Specific treatment should be classified according to different etiological factors, different treatment schemes should be chosen, and individual treatment should be carried out.

Key words: pulmonary alveolar proteinosis, treatment, whole lung lava