Pulmonary alveolar proteinosis is a rare disease in which a type of protein builds up in the air sacs (alveoli) of the lungs, making breathing difficult.
In some cases, the cause of pulmonary alveolar proteinosis is unknown. In others, it is associated with infection or immune deficiency.
This rare disorder generally affects people 30 - 50 years old and is seen in men more often than in women.
The doctor may hear crackles in the lungs. Often, the physical examination is normal.
Tests may reveal the following problems:
Treatment consists of periodically washing out the protein substance from the lung (whole-lung lavage). A lung transplant may be recommended for certain patients with this disease.
Some people with this condition go into remission, while others develop progressive respiratory failure.
Call your health care provider if you develop symptoms. Progressively worsening shortness of breath may signal that your condition is developing into a more serious medical emergency.
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Tamul PC, Peruzzi WT. Assessment and management of patients with pulmonary disease. Crit Care Med. April 2004; 32:S137-S145.