PERSISTENT
COUGH
PERSISTENT
COUGH
SHORTNESS
OF BREATH
SHORTNESS
OF BREATH
OF BREATH
FATIGUE
FATIGUE
If you have these common respiratory symptoms and are struggling to find an accurate diagnosis, you’re probably feeling confused and frustrated.
A rare lung disease may be the cause.
Autoimmune pulmonary alveolar proteinosis (aPAP) is a lung disease that is hard to diagnose and often misdiagnosed. This is because its symptoms are also common to other respiratory illnesses like pneumonia, bronchitis, and asthma.
A simple, accurate, and noninvasive blood test can help confirm or rule out aPAP at no cost to you or your doctor.
Do you have respiratory symptoms that
won't go away, even after treatment?
Common signs and symptoms
(including difficulty climbing stairs, walking)
(weight loss or weight gain due to limited activity)
If you've experienced any of these symptoms with no relief from treatment, and your doctor has mentioned unusual patterns in your lung scans—it may be time to talk to them about aPAP.
Because aPAP is a disease with symptoms that mimic more common respiratory conditions, it can take an average of 18 months from the time a person starts to feel symptoms to be diagnosed.
What is aPAP?
Pulmonary alveolar proteinosis (PAP) is a lung disease in which a substance called surfactant builds up in the lungs. Surfactant is a substance that may have an oily, mucus-like consistency. When surfactant builds up, it’s hard to breathe.
aPAP is the most common form of PAP, and it makes up about 90% of PAP cases. In aPAP, the immune system mistakenly targets the protein that activates the cells needed to keep the lungs clear.
In the United States, about 7 in 1 million people are diagnosed with aPAP.
However, because many doctors are not familiar with aPAP, they don’t even know to test for it—making the true number of aPAP cases unknown.
Although symptoms of aPAP can appear as early as childhood, most people are diagnosed in their 30s to 50s. It’s not more common in any sex, race, or region of the world. Studies suggest that smoking and exposure to fumes may contribute to an increased risk, but these are not always factors in people who are diagnosed.
How does aPAP affect you?
In aPAP, some white blood cells in the air sacs of the lungs (the alveoli) don’t work properly.
For these cells to work appropriately, they need a special protein called granulocyte-macrophage colony-stimulating factor (GM-CSF). But in aPAP, the body mistakenly blocks GM-CSF from binding to the white blood cells, preventing them from doing their job. As a result, surfactant—a substance with an oily, mucus-like consistency—builds up, which makes it hard to breathe. This blocking is caused by antibodies produced by the immune system, called GM-CSF autoantibodies.
However, since many pulmonologists are not familiar with testing for aPAP specifically, they may suggest these other tests first. These tests are more invasive and may require hospitalization.
Bronchoscopy
In a bronchoscopy, a doctor is able to see inside and examine a patient’s lungs and airways by passing a thin tube through the nose or mouth.
Biopsy
A biopsy may be performed by removing lung tissue or cells from the body. This can be done with a special needle or through surgery in an operating room.
How to rule out or confirm aPAP
One simple test
aPAP ClearPath™ is a simple, accurate, and noninvasive blood test that will help confirm or rule out autoimmune pulmonary alveolar proteinosis (aPAP), at no cost to you or your doctor.
The aPAP ClearPath™ blood test is available for your doctor to order as a serum-based test:
Talk to your doctor if you suspect you have aPAP
Start the conversation
When is the right time to talk to your doctor? Here are some questions that may help you decide:
- Have you been experiencing persistent cough, shortness of breath, and/or fatigue, and your condition doesn’t get better with treatment?
- Did your doctor mention that your last computerized tomography (CT) scan was abnormal?
- Are your daily activities, like walking to the mailbox or up the stairs, becoming harder?
While talking to your doctor about this may feel overwhelming, it doesn’t have to be. We’ve put together a few tips to help guide the conversation with your doctor.
- Make a list of symptoms and tests that you’ve had done so far. Having this to reference will make it easier to start the conversation.
- Consider bringing a family member for support.
- Take notes during your appointment and don’t be afraid to ask questions.
Here are a few resources that can help you find more
information about aPAP
The PAP Foundation
The PAP Foundation is a nonprofit patient advocacy organization dedicated to finding a cure and to improving the lives of those affected by pulmonary alveolar proteinosis (PAP). Through advocacy and collaboration, they promote research on PAP and provide information and support for PAP patients, healthcare professionals, and the public.
National Organization for Rare Disorders (NORD)
NORD builds community and creates a lasting impact by improving the health and well-being of people with rare diseases by driving advances in care, research, and policy. Their website includes a Pulmonary Alveolar Proteinosis Rare Disease Report and Video describing signs and symptoms, diagnosis, standard therapies, and more.
American Lung Association
Founded more than 120 years ago, the American Lung Association is the leading organization working to save lives by improving lung health and preventing lung disease through education, advocacy, and research. They have a section of their website dedicated to disease information about pulmonary alveolar proteinosis, including symptoms and diagnosis, treatment and management, and information for the newly diagnosed.
How to Find a Pulmonologist
The American Lung Association is a nonprofit patient advocacy organization with the mission to save lives by improving lung health and preventing lung disease. This section of their website is about how pulmonologists can help and how to find the right one for you.
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