Pulmonary alveolar proteinosis
PAP; Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis; Alveolar lipoproteinosis phospholipidosisPulmonary alveolar proteinosis (PAP) is a rare disease in which a type of protein builds up in the air sacs (alveoli) of the lungs, making breathing difficult. Pulmonary means related to the lungs.
Causes
In some cases, the cause of PAP 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 most often affected. PAP is seen in men more often than in women. A form of the disorder is present at birth (congenital).
Symptoms
Symptoms of PAP may include any of the following:
- Shortness of breath
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Cough
Cough
Coughing is an important way to keep your throat and airways clear. But too much coughing may mean you have a disease or disorder. Some coughs are d...
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Fatigue
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
Read Article Now Book Mark Article - Fever, if there is lung infection
- Bluish skin (cyanosis) in severe cases
Cyanosis
A bluish color to the skin or mucous membrane is usually due to a lack of oxygen in the blood. The medical term is cyanosis.
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Weight loss
Weight loss
Unexplained weight loss is a decrease in body weight, when you did not try to lose the weight on your own. Many people gain and lose weight. Uninten...
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Sometimes, there are no symptoms.
Exams and Tests
Your health care provider 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:
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Bronchoscopy with saline wash of the lungs (lavage)
Bronchoscopy
Bronchoscopy is a test to view the airways and diagnose lung disease. It may also be used during the treatment of some lung conditions.
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Chest x-ray
Chest x-ray
A chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.
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CT scan of the chest
CT scan of the chest
A chest CT (computed tomography) scan is an imaging method that uses x-rays to create cross-sectional pictures of the chest and upper abdomen....
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Lung function tests
Lung function tests
Pulmonary function tests are a group of tests that measure breathing and how well the lungs are functioning.
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Open lung biopsy (surgical biopsy)
Open lung biopsy
An open lung biopsy is surgery to remove a small piece of tissue from the lung. The sample is then examined for cancer, infection, or lung disease....
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Treatment
Treatment involves washing out the protein substance from the lung (whole-lung lavage) from time to time. Some people 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 people with alveolar proteinosis.
Support Groups
More information and support for people with pulmonary alveolar proteinosis and their families can be found at:
- National Organization for Rare Disorders -- rarediseases.org/rare-diseases/pulmonary-alveolar-proteinosis
- PAP Foundation -- www.papfoundation.org/
Outlook (Prognosis)
Some people with PAP go into remission. Others have a decline in lung function (respiratory failure) that gets worse, and they may need a lung transplant.
Respiratory failure
Respiratory acidosis is a condition that occurs when your lungs can’t remove all of the carbon dioxide produced by your body. This causes the blood ...
When to Contact a Medical Professional
Call your 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-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 85.
Trapnell BC, Mccarthy C. Pulmonary alveolar proteinosis syndrome. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 98.
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Respiratory system - illustration
Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.
Respiratory system
illustration
Review Date: 5/3/2023
Reviewed By: Denis Hadjiliadis, MD, MHS, Paul F. Harron, Jr. Professor of Medicine, Pulmonary, Allergy, and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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