摘要自身免疫性肺泡蛋白沉积症（autoimmune pulmonary alveolar proteinosis，aPAP）与机体内产生抗粒细胞-巨噬细胞集落刺激因子（granulocyte-macrophage colony-stimulating factor，GM-CSF）抗体有关，是肺泡蛋白沉积症最常见的类型，约占90%。重组人GM-CSF雾化吸入可有效治疗aPAP，但尚未获得我国批准，属于超适应证和超给药途径给药。肺泡蛋白沉积症共识专家组采用推荐分级的评估、制定和评价（grading of recommendation assessment，development and evaluation，GRADE）方法系统评估国内外专家应用GM-CSF雾化吸入治疗aPAP的经验，内容包括GM-CSF雾化吸入治疗aPAP的适用人群、GM-CSF雾化吸入治疗的给药方案、妊娠和哺乳期患者用药、药物不良反应和使用GM-CSF雾化吸入治疗的注意事项，并给出循证推荐建议。本专家共识有助于规范我国GM-CSF雾化吸入药治疗的临床应用，促进aPAP相关基础与临床研究。

abstractsAutoimmune pulmonary alveolar proteinosis (aPAP) is the most common(around 90%) type of pulmonary alveolar proteinosis, characterized by the presence of granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibody. Accumulating evidence has demonstrated the effectiveness of inhaled recombinant human GM-CSF for aPAP. Such a therapy has not been approved in China yet and has been classified off-label and unusual route of administration. Our expert consensus group evaluated the current knowledge with the methods of grading of recommendation assessment, development and evaluation (GRADE). The current evidence-based consensus statement covers the suitable population for inhaled GM-CSF therapy, details of prescription, usage during pregnancy and lactation, adverse effects and other suggestions in clinical usage. This consensus statement provides a clinical practice guideline of inhaled GM-CSF for aPAP in China and hopefully will accelerate basic and clinical research of aPAP.