Pulmonary Alveolar Proteinosis.

Pulmonary alveolar proteinosis (PAP) is a rare syndrome due to a number of underlying disorders, leading to accumulation of surfactant proteins in the alveoli and respiratory insufficiency. The diagnostic workup is aimed at identifying the underlying cause. Detection of GM-CSF neutralizing autoantibodies in serum confirms autoimmune PAP and rules out other causes. Whole lung lavage can effectively restore the alveolar function, but not cure the disease. Inhalational GM-CSF has shown to improve the disease outcome in autoimmune PAP, while treating the underlying disease is crucial in secondary PAP. Targeted treatment is the future in PAP caused by gene mutations.