Select your most common symptom

Have you been treated for

PERSISTENT
COUGH

but you're still coughing up a storm?

COUGH

Have you been treated for

SHORTNESS
OF BREATH

but every staircase feels like a mountain?

SHORTNESS
OF BREATH

Have you tried to manage your

FATIGUE

but you're still dragging yourself around?

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.

Pulmonary alveolar proteinosis (PAP) is a lung disease that's hard to diagnose and often misdiagnosed. This is because its symptoms are also common to other respiratory illnesses like pneumonia, bronchitis, and asthma.

Stay informed

Do you have respiratory symptoms that
won't go away, even after treatment?

PAP could be behind this.

Common signs and symptoms

These common respiratory symptoms could be caused by PAP:

Persistent cough

Shortness of breath

Overwhelming fatigue

(including difficulty climbing stairs, walking)

Unintentional changes in weight

(weight loss or weight gain due to limited activity)

Chest or back pain

Suddenly feeling out of shape or the need to rest during daily activities

Consistently feeling unwell

These symptoms are also common in other respiratory illnesses like pneumonia, bronchitis, and asthma, making PAP harder to diagnose.

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 PAP.

What is PAP?

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.

Types of PAP

Primary PAP: Caused by a disruption to specific white blood cells in your lungs that causes them to not work properly. This can fall into two categories:
- Autoimmune PAP (aPAP)
- Hereditary PAP
Secondary PAP: Caused by another disease (such as myelodysplastic syndrome or chronic myelogenous leukemia), certain infections, and potentially harmful toxins when inhaled (such as silica and talc)
Congenital PAP: The least common type of PAP and is the result of a genetic mutation, which occurs at birth

In the United States, about 7 in 1 million people are diagnosed with PAP.

However, because many doctors are not familiar with PAP, they don’t even know to test for it—making the true number of PAP cases unknown.

Autoimmune PAP (aPAP) is the most common form of PAP, accounting for about 90% of PAP cases.

In aPAP, the immune system mistakenly targets the protein that activates the cells needed to keep the lungs clear.

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.

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 correctly, 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.

A simple blood test can detect these GM-CSF autoantibodies to confirm or rule out aPAP. However, since many pulmonologists aren't 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.

Talk to your doctor about GM-CSF autoantibody blood testing

How to rule out or confirm aPAP

Savara Inc. sponsors a no charge, third-party testing program to reduce barriers to GM-CSF autoantibody testing. This program can also provide you and your healthcare professional with more information about how testing may be used as a diagnostic resource to help confirm or rule out aPAP and potentially reduce delays in diagnosis. Neither you, your healthcare provider, nor your health insurance provider will be billed for this program.

One simple test

Through the aPAP ClearPath™ Testing Program, a simple, accurate, and noninvasive blood test can help confirm or rule out aPAP.

This test is designed to detect the autoantibodies that cause aPAP.

This blood test is available for your doctor to order in 1 of 2 forms:

Serum-based test:

Blood is drawn from a vein into a blood tube using a needle.

Dried blood spot test:

Blood is drawn by a finger prick, then dropped directly onto a collection card.

Talk to your doctor to see if GM-CSF autoantibody blood testing is right for you.

Healthcare professionals and patients who use this program have no obligation to recommend, purchase, order, prescribe, promote, administer, use, or support any Savara product.

The aPAP ClearPath™ Testing Program is not intended to and should not interfere in any way with your healthcare professional’s or your independent judgment and choice in the management options for aPAP. Healthcare professionals and patients should always consider the full range of management options and select those most appropriate for the individual patient.

Living with aPAP

Many patients go months without knowing what's causing their persistent respiratory symptoms. This was the case for Eric, who lived with uncertainty until his doctor tested for aPAP. Now he's learning to navigate life after his diagnosis.

Meet Eric. A personal trainer whose diagnosis came as a surprise to him and his loved ones.

[Music]

[Male host]
It’s a rare, life-threatening lung disorder that impacts seven out of every 1 million people in the general population.

[Female host]
It occurs when an oily substance, normally present in the air sacs, builds up and causes a feeling of breathlessness that can worsen over time.

[Male host]
We'll learn more about this complex disease from a renowned pulmonologist. But first let's meet Eric, whose diagnosis came as a complete shock to this former personal trainer.

[Female host]
We're going behind the mystery of autoimmune pulmonary alveolar proteinosis, or aPAP.

[Eric]
After I was in the hospital for five or six days, things weren't looking really good. I could barely breathe, and nothing was working to clear out my lungs at all. The doctor told me that I had a 3% chance to live, and I need to say goodbye to my kids, so that was hard.

[Music]

[Eric]
I've always been really active from a young age. I started ice hockey as soon as I could walk. After I graduated college, I started working as a personal trainer, and fitness was a huge part of my life.

My kids were really young and we were moving to our dream house, and I moved everything just myself. And I noticed as I was moving, just walking up one flight of stairs, I was completely winded, which is very uncharacteristic of me.

Shortly after moving into that house, we took the kids down to Florida and I blew my knee out the first day, and when I got back home, I went to my orthopedist, who ordered a chest x-ray before he would operate on me. And I got a call on my anniversary evening, ready to go out to dinner, and the radiologist told me that my lung x-rays came back very abnormal and basically said I needed to see a pulmonologist as soon as possible.

[Music]

[Bruce C. Trapnell, MD]
Pulmonary alveolar proteinosis is a rare syndrome in which an oily substance called surfactant builds up in the air sacs of the lung, also called alveoli, and prevents oxygen from passing into the blood. Normally, surfactant is present in the air sacs as a thin layer that helps them stay open and function, and is normally cleared by alveolar macrophages.

Autoimmune PAP occurs when the immune system produces a protein called GMSCF autoantibody which blocks macrophages from functioning and prevents them from clearing surfactant from the air sacs.

The symptoms of autoimmune PAP are nonspecific and include breathlessness, cough, fatigue, occasionally white frothy sputum, chest congestion, chest pain, and some patients also have fever and cough up blood, which indicates that infection of the lung is also present.

[Eric]
As soon as I got to my appointment, the doctor was going over a sheet from the radiologist, and the radiologist said on there, “Looks like he has PAP,” and the pulmonologist looked at his diagnosis and looked at me and said, “There's absolutely no way you have PAP. You're way too healthy for that,” and then he ordered the bronchoscopy so he can confirm exactly what I had.

I went through that procedure. Everything seemed to go fine until later on that night, my chest started getting really, really heavy. I let it go for a few more hours and I generally just could not breathe at all.

So, we hopped in the car and rushed off to the hospital and by 2:30 in the morning, my lungs were collapsing, and I had double pneumonia. I was on every kind of antibiotic you could imagine, and nothing was getting better. The doctor told me I had a 3% chance to live, and I should probably say my goodbyes to my family.

Some of my friends had gone in and started researching what the radiologist had originally said, PAP, and they found Dr Trapnell up at Cincinnati Children's. Dr Trapnell suggested that they do the blood test to confirm or rule out whether or not I in fact have PAP. Once the blood test came back, it showed that I did in fact have autoimmune PAP.

[Music]

[Bruce C. Trapnell, MD]
The diagnosis of autoimmune PAP is challenging because the symptoms are nonspecific. They occur in other very common lung diseases, and physical findings are generally minimal while routine laboratory tests are frequently normal. Most patients are diagnosed initially incorrectly as having pneumonia as adults, or as having asthma as children.

Historically, a lung biopsy has been used to diagnose PAP. However, lung biopsies are unable to diagnose any specific PAP-causing disease including autoimmune PAP.

In marked contrast, a simple blood test to measure the level of GM-CSF autoantibodies can provide a definitive diagnosis of autoimmune PAP and exclude other lung diseases.

Had Eric undergone blood testing for suspected autoimmune PAP, the lung biopsy and subsequent infection and hospitalization would have been avoided.

[Eric]
After a few months went by, I had to travel up to Cincinnati Children’s to meet Dr Trapnell for the first time. I think I ran up to the guy and hugged him.

I'd never met him in my life, but the man truly saved my life. He took as much time as we needed to understand what autoimmune PAP was.

[Music]

[Bruce C. Trapnell, MD]
Currently, there is no cure for autoimmune PAP. The treatment strategy depends on the severity of the disease and, generally, patients are currently treated with whole lung lavage, which requires hospitalization, general anesthesia, mechanical ventilation—which is a breathing machine—and requires a team to perform.

In whole lung lavage, one lung is connected to a breathing machine while the other is repeatedly filled with salt water and drained to physically wash surfactant out of the lung. Whole lung lavage can be effective, but it does not stop the disease, does not stop surfactant accumulation, and is typically required periodically by most patients.

[Music]

[Eric]
After I received the first lung lavage, I was night and day better. You realize how much oxygen you've been missing. It's amazing. I felt like I was 20 years old again.

After that, they did a lavage on my second lung, and then it was about every 8 months or so. I'd have to keep going back in. It would cause a lot of anxiety because you know you're going under anesthesia, and then the pain you feel in your lungs afterwards. That's not ideal at all for treatment.

If my lungs were getting clogged, my energy levels were really low, and it was a struggle breathing. After going through a few lung lavages, it became pretty apparent to me that we need more options in terms of managing autoimmune PAP.

[Bruce C. Trapnell, MD]
The goals of treatment in patients with PAP are to reduce symptoms, improve oxygenation, and to improve quality of life—ultimately to address the cause of the disease. My advice for patients living with autoimmune PAP is to learn about the disease. Understanding the disease can help patients make better choices when needed.

The patient should talk to their doctor about their symptoms and their ability to perform their normal activities of daily living. It's useful to connect to other patients with similar problems and there are organizations, for example, the PAP Foundation, that can help provide patients with autoimmune PAP with help and information.

[Eric]
Now that I know I have autoimmune PAP, life is similar, just a little bit slower, and I have to constantly manage my oxygen levels in my lungs. I spend time with my kids, try to keep up with them. Over the years that I've known I've had autoimmune PAP, I've been able to connect with other people with the same disease, and it was kind of nice to know that I'm not the only one and that there are other people out there.

I suggest for anyone with autoimmune PAP that they reach out to their pulmonologist, and hopefully you can get someone really good like Dr Trapnell, and get your blood tested and you have your answer right away.

Don't wait on it. Don't wait on it at all.

[Music]

[Male host]
If you or someone you know has any of the signs or symptoms of autoimmune PAP, ask your pulmonologist to order a simple, accurate, noninvasive, no-cost blood test at apapclearpath.com.

[Female host]
If you have been diagnosed with pulmonary alveolar proteinosis, or PAP, visit papfoundation.org. You could always go to our website, thebalancingact.com.

[Male host]
And we'll be back right after this.

[Music]

If you, like Eric, find yourself with persistent respiratory symptoms that will not go away with treatment, talk to your doctor about testing for aPAP with a blood test. You can learn more and stay up to date on aPAP by signing up for updates.

Talk to your doctor if you suspect you have aPAP

If you think you may have autoimmune pulmonary alveolar proteinosis (aPAP), it’s important to find a pulmonologist to diagnose and manage the disease properly.

Because aPAP is so rare, many doctors may have never diagnosed or treated aPAP. If you’ve been struggling with your ongoing symptoms after other lung-related diagnoses, it may be time to talk with your pulmonologist about 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.

Prepare for your next appointment by filling out the notes page below.Download

If your pulmonologist wants to learn more, share the link to aPAPClearPath.com where they can get more information about PAP and the aPAP ClearPath™ Testing Program.Healthcare Professional Site

Here are a few resources that can help you find more
information about PAP

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.

Find tools and support

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.

Build your community

American Lung Association

Founded more than 120 years ago, the American Lung Association is the leading organization working to save lives by improving 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.

Find more resources

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.

Find a pulmonologist

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If you have these common respiratory symptoms and are struggling to find an accurate diagnosis, you’re probably feeling confused and frustrated.

Do you have respiratory symptoms that
won't go away, even after treatment?

Common signs and symptoms

What is PAP?

Types of PAP