1 Department of Respiratory Medicine and.
2 Department of Occupational Health, Graduate School of Medicine, Tohoku University, Sendai, Japan.
Ann Am Thorac Soc. 2017 Aug;14(8):1298-1304. doi: 10.1513/AnnalsATS.201611-892BC.
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by the excessive accumulation of surfactant proteins within the alveolar spaces and by higher titers of autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF) in the serum and bronchoalveolar lavage fluid. The antibodies inhibit the maturation and phagocytosis of alveolar macrophages. Although the standard therapy for aPAP has been whole-lung lavage (WLL), this procedure is invasive and needs to be repeated for several years. GM-CSF inhalation therapy is a new procedure for treating aPAP and can induce remission with less invasiveness, although it is generally less effective in severe cases. We evaluated five cases with remarkable improvement by using sequential GM-CSF inhalation therapy after WLL; however, the treatment failed when this therapy preceded WLL. Therefore, sequential GM-CSF inhalation after WLL may reinforce the efficiency of WLL in patients with severe aPAP.
自身免疫性肺肺泡蛋白沉积症（aPAP）是一种罕见疾病，其特征是肺泡腔内表面活性剂蛋白过度积聚，以及血清和支气管肺泡灌洗液中粒细胞-巨噬细胞集落刺激因子（GM-CSF）自身抗体滴度较高。这些抗体抑制肺泡巨噬细胞的成熟和吞噬作用。尽管全肺灌洗（WLL）是 aPAP 的标准治疗方法，但该操作具有侵袭性，需要多年重复进行。GM-CSF 吸入疗法是一种新的治疗 aPAP 的方法，其侵袭性较小，但在严重病例中效果通常较差。我们评估了五例患者在 WLL 后序贯 GM-CSF 吸入治疗后显著改善的病例；然而，当这种疗法先于 WLL 时，治疗失败。因此，WLL 后序贯 GM-CSF 吸入可能会增强严重 aPAP 患者 WLL 的疗效。
