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

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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.