Li Meirui, Alowami Salem, Schell Miranda, Davis Clive, Naqvi Asghar
Michael G. DeGroote School of Medicine, McMaster University, Hamilton, ON, Canada.
Department of Pathology and Molecular Medicine, McMaster University, Hamilton, ON, Canada.
Case Rep Pulmonol. 2018 Jan 23;2018:5202173. doi: 10.1155/2018/5202173. eCollection 2018.
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which defects in alveolar macrophage maturation or function lead to the accumulation of proteinaceous surfactant in alveolar space, resulting in impaired gas exchange and hypoxemia. PAP is categorized into three types: hereditary, autoimmune, and secondary. We report a case of secondary PAP in a 47-year-old man, whose risk factors include occupational exposure to inhaled toxins, especially aluminum dust, the use of anabolic steroids, and alcohol abuse, which in mice leads to alveolar macrophage dysfunction through a zinc-dependent mechanism that inhibits granulocyte macrophage-colony stimulating factor (GM-CSF) receptor signalling. Although the rarity and vague clinical presentation of PAP can pose diagnostic challenges, clinician awareness of PAP risk factors may facilitate the diagnostic process and lead to more prompt treatment.
肺泡蛋白沉积症（PAP）是一种罕见的肺部疾病，其中肺泡巨噬细胞成熟或功能缺陷导致肺泡腔内蛋白质性表面活性剂积聚，从而导致气体交换受损和低氧血症。PAP分为三种类型：遗传性、自身免疫性和继发性。我们报告一例47岁男性继发性PAP病例，其危险因素包括职业性接触吸入毒素，尤其是铝尘、使用合成代谢类固醇和酗酒，在小鼠中，这些因素通过抑制粒细胞巨噬细胞集落刺激因子（GM-CSF）受体信号传导的锌依赖性机制导致肺泡巨噬细胞功能障碍。尽管PAP的罕见性和模糊的临床表现可能带来诊断挑战，但临床医生对PAP危险因素的认识可能有助于诊断过程并导致更及时的治疗。
