Pulmonary Alveolar Proteinosis (PAP) and Inhaled Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) Therapy--ClinicalFeatures Predicting Response and Recurrence.

自身免疫性肺泡蛋白沉积症(APAP)是由肺内粒细胞/巨噬细胞集落刺激因子(GM-CSF)自身抗体引起的。此前，我们报道了GM-CSF吸入治疗改善了这些患者的肺泡-动脉氧分压差和疾病严重程度的血清生物标志物。为了找出GM-CSF吸入治疗APAP的预测和预后因素，我们对APAP患者吸入GM-CSF治疗前后的BALF进行了回顾性分析。结果表明，吸入GM-CSF可降低自身免疫性肺泡蛋白沉积症患者BALF中GM-Ab的含量，降低BALF中总蛋白和SP-A的含量，升高IL-17和肿瘤抗原-125的含量，这可能与肺清除功能恢复有关。此外，通过治疗后30个月的观察，我们得出结论，吸入GM-CSF治疗的患者有一半以上持续缓解，基线%VC可能是疾病复发的预后因素。

Autoimmune pulmonary alveolar proteinosis (aPAP) is caused by granulocyte/macrophage-colony stimulating factor (GM-CSF) autoantibodies in the lung. Previously, we reported that GM-CSF inhalation therapy improved alveolar-arterial oxygen difference and serum biomarkers of disease severity in these patients. To figure out predictive and prognostic factors for GM-CSF inhalation therapy in aPAP, we performed retrospective analyses of BALF obtained in aPAP patients before and after GM-CSF inhalation therapy. We confirmed that GM-CSF inhalation was associated with a decrease of GM-Ab in the BALF in improved lungs, which was probably due to the restoration of clearance, and that GM-CSF inhalation decreased the concentration of total protein and SP-A in BALF, and increase interleuki-17 and cancer antigen-125 in improved lung of autoimmune pulmonary alveolar proteinosis. Furthermore, through the post-treatment, 30-month observation, we concluded that inhaled GM-CSF therapy sustained remission in more than half of cases, and baseline %VC might be a prognostic factor for disease recurrence.