Response to inhaled granulocyte-macrophage colony-stimulating factor in patient with mild-to-moderate autoimmune pulmonary alveolar proteinosis-24 months of follow-up.

Abstract

Autoimmune pulmonary alveolar proteinosis (aPAP) is a disease caused by IgG antibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). Whole lung lavage (WLL) allows to remove the lipo-proteinaceous material accumulated by the poor clearance of alveolar surfactant. However, it is a complex technique that is not exempt from complications, and in some cases, the patients are refractory, requiring the performance of several WLLs spaced apart in time. We present the clinical, functional, and radiological evolution after 24 months of follow-up of a patient diagnosis of aPAP refractory to WLL, with performed three therapeutic WLLs spaced 16 and 36 months and serious potentially fatal complications in the last one. After 24 months, no adverse effects have appeared and the great clinical, functional and radiological response is maintained. The patient has beensuccessfully treated with inhaled recombinant human GM-CSF sargramostim.