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Alternative Names Return to topAlveolar proteinosis
Definition Return to top
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.
Causes Return to top
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 Return to top
Exams and Tests Return to top
The doctor may hear crackles in the lungs. Often, the physical examination is normal.
Tests may reveal the following problems:
Treatment Return to top
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.
Outlook (Prognosis) Return to top
Some people with this condition go into remission, while others develop progressive respiratory failure.
When to Contact a Medical Professional Return to top
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 Return to top
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.Update Date: 3/1/2007 Updated 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.