Pulmonary alveolar proteinosis (PAP; the accumulation of surfactant lipids
and proteins in the alveoli) has a number of infectious and environmental c
auses but is usually idiopathic. The clinical presentation of PAP is nonspe
cific; thus, the diagnosis is frequently missed, leading to inappropriate t
herapy and unnecessary morbidity. Recent advances suggest that a deficiency
in granulocyte-macrophage colony-stimulating factor (GM-CSF) activity may
lead to this surfactant accumulation. Anti-GM-CSF antibodies have been foun
d in PAP patients, fueling speculation that PAP may be an autoimmune diseas
e. These findings are being translated into novel forms of therapy.