Khan Shana N S, Stirling Robert G, Mclean Catriona A, Russell Prudence A, Hoy Ryan F
Department of Respiratory Medicine Alfred Health Melbourne Victoria Australia.
Department of Medicine Monash University Melbourne Victoria Australia.
Respirol Case Rep. 2022 Aug 11;10(9):e01021. doi: 10.1002/rcr2.1021. eCollection 2022 Sep.
Pulmonary alveolar proteinosis (PAP) is a rare lung disease where there is accumulation of surfactant in the alveoli. It can be classified based on the underlying aetiology into three categories: primary, secondary and congenital. Autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF-Ab) are a key diagnostic feature of autoimmune PAP. High intensity occupational exposure and inhalation of toxic particles such as silica can cause a form of secondary PAP called acute silicoproteinosis. We describe a 26-year-old stone benchtop fabricator with silicoproteinosis following daily exposure to high levels of silica who had elevated serum GM-CSF-Ab. We discuss the role of GM-CSF-Ab in cases of PAP with occupational inhalational exposure and the challenges in its interpretation.
肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病,其特征是肺泡内表面活性物质积聚。根据潜在病因,它可分为三类:原发性、继发性和先天性。抗粒细胞-巨噬细胞集落刺激因子自身抗体(GM-CSF-Ab)是自身免疫性PAP的关键诊断特征。高强度职业暴露和吸入有毒颗粒(如二氧化硅)可导致一种称为急性硅蛋白沉积症的继发性PAP。我们描述了一名26岁的石材台面加工工人,他在每天接触高浓度二氧化硅后患上了硅蛋白沉积症,其血清GM-CSF-Ab水平升高。我们讨论了GM-CSF-Ab在职业性吸入暴露所致PAP病例中的作用及其解读方面的挑战。