Skip to main content

Health Encyclopedia

Search the Health Encyclopedia

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; Pulmonary alveolar phospholipoproteinosis

Causes

In some cases, the cause of pulmonary alveolar proteinosis is unknown. In others, it occurs with lung infection or an immune problem. It also can occur with cancers of the blood system, and after exposure to high levels of environmental substances, such as silica or aluminum dust.

People between 30 and 50 years old are affected. The condition is seen in men more often than in women. A form of the disorder is present at birth (congenital).

Symptoms

Symptoms may include any of the following:

Sometimes there are no symptoms.

Exams and Tests

The doctor will listen to the lungs with a stethoscope and may hear crackles (rales) in the lungs. Often, the physical examination is normal.

The following tests may be done:

Treatment

Treatment involves washing out the protein substance from the lung (whole-lung lavage) from time to time. Some persons may need a lung transplant. Avoiding dusts that might have caused the condition is also recommended.

Another treatment that may be tried is a blood-stimulating drug called granulocyte-macrophage colony stimulating factor (GM-CSF), which is lacking in some persons with alveolar proteinosis.

Outlook (Prognosis)

Some people with this condition go into remission. Others have respiratory failure that gets worse, and they may need a lung transplant. Up to 25% of people with this condition die within 5 years of being diagnosed.

When to Contact a Medical Professional

Call your health care provider if you develop serious breathing symptoms. Shortness of breath that gets worse over time may signal that your condition is developing into a medical emergency.

References

Levine SM. Alveolar filling disorders. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 91.

Trapnell BC, Luisetti M. Pulmonary alveolar proteinosis syndrome. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 70.

Review Date:6/22/2015
Reviewed By:Denis Hadjiliadis, MD, MHS, Associate Professor of Medicine, Pulmonary, Allergy, and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997-A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

adam.com

The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.

Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.

Health
Outcome Data

No data available for this condition/procedure.

Health Encyclopedia

More Features

We Appreciate Your Feedback!
1. Did you find this information useful?
         Yes
         No
2. Would you recommend this website to family and friends?
         Yes
         No