Abstract:
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.
摘要:
肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病,其中肺泡中存在表面活性物质的积累。它可以根据潜在的病因分为三类:原发性,继发性和先天性。粒细胞-巨噬细胞集落刺激因子(GM-CSF-Ab)的自身抗体是自身免疫性PAP的关键诊断特征。高强度职业暴露和吸入二氧化硅等有毒颗粒可导致一种称为急性硅蛋白病的继发性PAP。我们描述了一名26岁的石头台式制造商,每天暴露于高水平的二氧化硅后,其血清GM-CSF-Ab升高。我们讨论了GM-CSF-Ab在职业性吸入暴露的PAP病例中的作用及其解释中的挑战。