自身免疫性肺泡蛋白沉积症与抗GM-CSF抗体

Autoimmune pulmonary alveolar proteinosis and anti-GM-CSF antibody

肺泡蛋白沉积症(PAP)是一种由于肺泡表面活性物质在肺泡巨噬细胞和肺泡腔内异常沉积导致呼吸困难为主要特征的弥漫性肺疾病,其中自身免疫型PAP(aPAP)是最常见的临床类型,约占90%。由于抗粒细胞-巨噬细胞集落刺激因子抗体(抗GM-CSF抗体)的异常产生,导致肺泡巨噬细胞功能障碍。本文讨论GM-CSF信号通路的异常、肺泡巨噬细胞的失能以及自身抗体的产生,阐明aPAP发病机制的研究现状,介绍aPAP的诊疗进展。

Pulmonary alveolar proteinosis(PAP)is a diffuse lung disease characterized by dyspnea due to abnormal deposition of pulmonary surfactant in alveolar macrophages and alveoli,among which autoimmune PAP(aPAP)is the most common clinical type,accounting for about 90%.The abnormal production of anti-granulocyte-macrophage colony stimulating factor antibody(anti-GM-CSF antibody)leads to the dysfunction of alveolar macrophages.We summarize the current state of research on the pathogenesis of aPAP and describe the progress in the diagnosis and treatment of aPAP by discussing the abnormalities of signal pathway,dysfunction of alveolar macrophages and the production of anti-GM-CSF antibody.