Advances in the pathogenesis and treatment of pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis is a rare condition characterized by accumulation of surfactant components, both proteins and phospholipids, within alveoli and distal airways, leading to impairment of gas exchange and eventually to respiratory failure in approximately one third of carriers. The natural history of the disease has been dramatically changed by the whole lung lavage. Studies performed using transgenic animals suggest that granulocyte-macrophage colony-stimulating factors may play a role in the development of the disease, thus opening the way to innovative therapeutic strategies.