Pulmonary alveolar proteinosis (PAP) is a rare
lung disease characterized by the accumulation of lipoproteinaceous material within the alveoli. Several studies have recently found that
autoantibodies against
granulocyte-macrophage colony-stimulating factor (
GM-CSF)
play a major
role in the pathogenesis of idiopathic PAP. Consequently, inhaled or systemic
injection of
GM-CSF has been suggested as a promising
treatment for PAP. A 54-year-old
male visited our
hospital for progressive
dyspnea. Four years earlier, he was diagnosed with PAP based on a surgical
lung biopsy in another institution. Whole-
lung lavage was performed four times before he visited our
hospital. We administered high-
dose inhaled
GM-CSF therapy for 12 weeks followed by 12 weeks of low-
dose therapy. After the
GM-CSF treatment, the
patient's symptoms,
lung function, and radiological findings were improved significantly.