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Pulmonary alveolar proteinosis

Contents of this page:

Illustrations

Respiratory system
Respiratory system

Alternative Names    Return to top

Alveolar 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

There may be no symptoms.

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.

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