What is pulmonary alveolar
Pulmonary alveolar proteinosis (PAP) is a rare lung condition. It occurs
when surfactant builds up in your lungs and clogs your air sacs, or alveoli. Surfactant
is a natural substance that lowers surface tension in your lungs and allows you
to breathe. The three main types of PAP are congenital, acquired, and
PAP causes mild to severe breathing problems, ranging from shortness of
breath with a lot of exertion to shortness of breath at rest. It can be fatal
if you don’t get treatment for a severe case of it.
are the symptoms of pulmonary alveolar proteinosis?
It’s possible to have PAP without having any symptoms. The symptoms of PAP
breathing, which is the most common symptom
occasionally with mucus or blood
- a blue-tinged
- general fatigue
- a low-grade
- weight loss
The symptoms of PAP can progress to severe lung impairment and respiratory
failure if you don’t get treatment for them.
What causes pulmonary alveolar proteinosis?
Surfactant coats your lungs. PAP develops when this substance reaches an
abnormal level and blocks your lung’s airways. This impairs the transfer of
oxygen from your lungs to your blood. It often results in labored breathing.
The exact cause of PAP is unknown.
What are the risk
factors for pulmonary alveolar proteinosis?
According to the PAP
Foundation, this condition affects roughly 3.7 people per million. This
makes it a rare disease. The majority of cases appear later in life. People
between the ages of 30 and 50 are more likely to develop the condition, and men
are at greater risk than women.
Cases of PAP may be linked to:
- an immune
response that blocks the breakdown of surfactant
- lung infection
- certain cancers
- exposure to
toxic particles in the environment or workplace, such as silica or
How is pulmonary alveolar proteinosis diagnosed?
If your doctor suspects you have PAP, they may perform the following tests:
- a chest X-ray to
look for white patches in your lungs
- a CT scan of your
chest to look for white patches in your lungs
- blood tests to
check for antibodies that are related to PAP
function tests to check the air capacity of your lungs
- a bronchoscopy
with saline wash to obtain a fluid sample from your lungs
- a lung biopsy to
examine a tissue sample from your lungs
is pulmonary alveolar proteinosis treated?
In some cases, the symptoms of PAP are so mild that no treatment is necessary.
The condition sometimes resolves without treatment. If you have certain mild
symptoms, supplemental oxygen therapy may be enough to treat the condition.
If you have severe symptoms, your doctor can wash the surfactant out of your
lungs with a saline solution. Depending on the area affected, they may wash
only part of your lung. If your entire lung needs to be cleaned, they’ll use a
procedure called “whole lung lavage.” In this procedure, they’ll fill one of
your lungs with saline solution and drain it, while keeping your other lung
One wash may be enough to clear your symptoms, but you’ll most likely need
multiple treatments. Your doctor may also prescribe a blood-stimulating
medication, which is a newer treatment that has improved symptoms for some
people. As a last resort, they may recommend a lung transplant.
with pulmonary alveolar proteinosis
It’s important to get treatment for PAP if your condition is severe. PAP is
fatal within five years of diagnosis for about 20
percent of people with the condition. The cause of death is usually
respiratory failure or lack of oxygen in the blood.
For other people, PAP is manageable with treatment. You can live a
relatively normal life after your doctor diagnoses you with PAP and treats it.
However, you may have still have some shortness of breath for years afterward.
You may have permanent scarring in your lungs and reduced lung capacity, but
this is uncommon. All people who develop PAP have an increased risk of
pneumonia, which is an infection in the lung.