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Pulmonary alveolar proteinosis
Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis
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
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:
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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.
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Shortness of breath
Shortness of breath
Breathing difficulty may involve:Difficult breathingUncomfortable breathingFeeling like you are not getting enough air
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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
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:
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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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High-resolution
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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Pulmonary function tests
Pulmonary 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 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.
Respiratory
The words "respiratory" and "respiration" refer to the lungs and breathing.
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.
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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: 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.
