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Pulmonary alveolar proteinosis

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

• Cough
• Unintentional weight loss
• Fatigue
• Shortness of breath
• Fever

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:

• Chest x-ray may show dense shadows similar to fluid build-up in the lungs (pulmonary edema).
• Arterial blood gases test may reveal low oxygen levels in the blood.
• Pulmonary function tests may show restrictive lung disease and abnormal diffusion.
• High-resolution CT scan of the chest may show dense infiltrates in both lungs.
• Bronchoscopy with saline wash of the lungs (lavage) may show a milky fluid.

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.

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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