If your doctor suspects PAP (Pulmonary Alveolar Proteinosis), a series of tests will be performed to confirm the diagnosis. These may include chest x-ray, chest CT, oxygen levels, and breathing tests. In addition, a bronchoscopy may be performed with washing to find excessive surfactant in the lungs. Lung biopsies may also be performed. Autoimmune PAP is diagnosed by detection of the antibody in the blood. Hereditary PAP is diagnosed through genetic testing.
Pulmonary Alveolar Proteinosis Treatment
Mild PAP often does not require treatment. When PAP results in significant shortness of breath and low oxygen levels, treatment is indicated. Historically, the principal treatment has been whole lung lavage, which means washing out the proteinaceous material from the lungs. This procedure generally results in improvement, although disease may recur, and multiple lavages may be necessary. Autoimmune PAP may be treated with inhaled GM-CSF. In severe cases that do not respond to standard therapy, immunosuppression may be considered. The treatment for secondary PAP is treatment of the underlying condition.
Autoimmune PAP generally has a favorable prognosis. Although some patients have progressive disease, many remain stable and a small percentage improve spontaneously. Secondary PAP generally has a worse prognosis but may resolve if the underlying condition can be effectively treated.