Pulmonary alveolar proteinosis

[Agnes]

Pulmonary alveolar proteinosis is a rare disorder in which the air sacs of the lungs (alveoli) become plugged with a protein- and fat-rich fluid.

Pulmonary alveolar proteinosis typically affects people who are aged 20 to 50 and who have not had lung disease.
People have difficulty breathing and cough.
Diagnosis is by computed tomography and testing a sample of lung fluid obtained using a bronchoscope.
If symptoms are severe, the lungs are washed out, one at a time.

The cause of pulmonary alveolar proteinosis is almost always unknown, but recent studies have linked it to production of an antibody directed against a protein that seems to be involved with the production or the breakdown of surfactant (a substance normally produced in the lungs). Occasionally, development of pulmonary alveolar proteinosis is related to exposure to toxic substances, such as inorganic dusts, infection with Pneumocystis jirovecii, certain cancers, and immunosuppressants. Rarely, it occurs in newborns.
The protein in the lungs plugs up the alveoli and small airways (bronchioles). In rare instances, lung tissue becomes scarred. The disease may progress, remain stable, or disappear spontaneously.