Definition:
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.
Alternative Names:
Alveolar proteinosis
Causes, incidence, and risk factors:
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.
Symptoms:
There may be no symptoms.
Signs and tests:
The doctor may hear crackles in the lungs. Often, the physical examination is normal.
Tests may reveal the following problems:
Treatment:
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.
Expectations (prognosis):
Some people with this condition go into remission, while others develop progressive respiratory failure.
Calling your health care provider:
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.
References:
Mason RJ, Broaddus VC, Murray JF, Nadel, JA. Murray and Nadel's Textbook of Respiratory Medicine. 4th ed. Philadelphia, Pa: WB Saunders; 2005.
Presneill JJ, Nakata K, Inoue Y, Seymour JF. Pulmonary alveolar proteinosis. Clin Chest Med. September 2004; 25:593-613.
Tamul PC, Peruzzi WT. Assessment and management of patients with pulmonary disease. Crit Care Med. April 2004; 32:S137-S145.
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Review Date:3/1/2007
Reviewed By:David A. Kaufman, M.D., Assistant Professor, Division of Pulmonary Medicine, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.
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