Although spontaneous resolution of PAP is described, most patients are treated with physical removal of the lipoproteinaceous material through large-volume lung lavage. Whole-lung lavage is highly effective in improving symptoms, oxygenation, and radiographic infiltrates, and likely has a survival benefit; however, symptoms and infiltrates often recur, and repeat lavages may be necessary. Autoimmune PAP can be treated with inhaled GM-CSF, which is effective in ∼69% of patients and generally is well tolerated. Secondary PAP usually responds to treatment of the underlying disease. The overall prognosis of primary PAP is favorable because the disease typically becomes quiescent to the extent that continued therapy is not necessary. Occasionally, pulmonary fibrosis may develop. The overall 5-year survival rate of primary PAP is upwards of 90%, but mortality is well described from PAP and its complications, such as CNS nocardiosis, which itself carries a mortality rate of up to 44%.