Kroll Ryan R, Kumar Sameer, Grossman Ronald F, Price Charles, Srigley John R
Department of Internal Medicine, Queen's University, Etherington Hall, Room 3033, 94 Stuart Street, Kingston, ON, Canada K7L 3N6.
Trillium Health Partners-Credit Valley Hospital Site, Mississauga, ON, Canada L5M 2N1.
Can Respir J. 2016;2016:4064539. doi: 10.1155/2016/4064539. Epub 2016 May 16.
Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by dysfunctional alveolar macrophages, which ineffectively clear surfactant and typically cause mild hypoxemia. Characteristic Computed Tomography findings are septal reticulations superimposed on ground-glass opacities in a crazy paving pattern, with a clear juxtaposition between affected and unaffected parenchyma. While traditionally PAP was diagnosed via biopsy, bronchoalveolar lavage (BAL) is usually sufficient; the fluid appears milky, and on microscopic examination there are foamy macrophages with eosinophilic granules and extracellular hyaline material that is Periodic Acid-Schiff positive. Standard therapy is whole lung lavage (WLL), although novel treatments are under development. The case presented is a 55-year-old woman with six months of progressive dyspnea, who developed hypoxemic respiratory failure requiring mechanical ventilation; she had typical findings of PAP on imaging and BAL. WLL was ultimately successful in restoring adequate oxygenation. Respiratory failure of this magnitude is a rare finding in PAP.
肺泡蛋白沉积症（PAP）是一种罕见疾病，其特征为肺泡巨噬细胞功能失调，这些巨噬细胞无法有效清除表面活性物质，通常会导致轻度低氧血症。计算机断层扫描的特征性表现为间隔网状影叠加在磨玻璃样混浊上，呈铺路石样改变，病变实质与未受累实质之间界限清晰。传统上，PAP通过活检进行诊断，但支气管肺泡灌洗（BAL）通常就足够了；灌洗液呈乳状，显微镜检查可见含有嗜酸性颗粒的泡沫状巨噬细胞和过碘酸希夫染色阳性的细胞外透明物质。标准治疗方法是全肺灌洗（WLL），不过新型治疗方法正在研发中。本病例为一名55岁女性，有6个月进行性呼吸困难病史，发展为需要机械通气的低氧性呼吸衰竭；她在影像学和BAL检查中有PAP的典型表现。WLL最终成功恢复了充足的氧合。如此严重的呼吸衰竭在PAP中是罕见的表现。
