286

To understand idiopathic pulmonary fibrosis (IPF) better, it’s important to understand how your lungs work. As you breathe in, the air goes from your mouth down your trachea. This is the tube that connects your nose and mouth to your lungs. Air flows down your trachea through your bronchial tubes into both your left and right lungs. Your bronchial tubes have many branches that look like an upside-down tree.

At the very ends of these branches, like berries at the end of a bush, are tiny air sacs called alveoli. Around the alveoli are capillaries—tiny blood vessels that eventually lead back to your heart. The alveoli allow oxygen from the air you breathe in to pass into your blood, where it’s stored in your red blood cells for use elsewhere in your body. The alveoli also allow carbon dioxide from your blood to be released back into the air.

How Your Lungs Work

In a normal lung, oxygen passes easily through the bronchial tubes to the alveoli, where it’s transferred to the bloodstream. (See illustration below.) In a person with IPF, the lungs can’t function correctly due to scarring (“fibrosis”). This scarring happens in the spaces between the air sacs, and it reduces the amount of oxygen the lungs can transfer into the bloodstream. This leads to shortness of breath and fatigue. As IPF progresses and lung function is reduced, shortness of breath worsens. Oxygen therapy is often needed to help patients with reduced lung function breathe more easily. (Learn more about what IPF is and how it affects your lungs.)

How Lungs Work

Air travels into the lungs via the windpipe (trachea). It goes down a series of branching airways called bronchi. These branch into smaller bronchioles and then into millions of tiny air sacs (alveoli).

Oxygen (O2) in the air passes through the thin walls of the alveoli into the tiny blood vessels nearby. Oxygen attaches to red blood cells and is carried in blood vessels to the rest of the body.

Carbon dioxide (CO2) is passed out of the alveoli from the red blood cells and is breathed out.

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229

If you think you could be suffering from idiopathic pulmonary fibrosis (IPF)—or even if you’ve already been diagnosed—you may feel confused and overwhelmed. We’re here to help you better understand what IPF is and how it impacts the body. Once you understand the condition, you’ll have the information you need to take an active role in managing it.

How Does IPF Impact the Lungs?

Healthy Lung vs. IPF Lung

Let’s explore what actually happens in the lungs of a person with IPF. Normally, oxygen in the air is inhaled through the mouth or nose and travels down the throat and into the lungs.

Once inside the lungs, oxygen passes through tiny air sacs (alveoli) before being released into the bloodstream. At the same time, carbon dioxide is passed from the bloodstream to the lungs to be exhaled. After oxygen is released into the bloodstream, it’s carried to other organs inside the body. These processes are necessary for survival.

In a person with IPF, however, the space between the air sacs and the blood vessels thicken with scar tissue, which leads to progressive lung destruction. As the air sac walls thicken, it becomes more difficult to inhale enough air and for oxygen to pass into the bloodstream. Eventually, these air sacs no longer work. When this happens, organs may not receive the oxygen they need to function properly. This lack of oxygen can lead to symptoms like shortness of breath, tiredness, and clubbing of the fingertips or toes, meaning they are rounded and widened.

What Happens as IPF Progresses?

Over time, the scarring of the lungs causes them to stiffen, making it difficult for the lungs to expand fully. Breathing becomes difficult when this happens. While IPF will eventually progress for everyone, it’s important to know that it may progress at different speeds for different people. For some, IPF worsens fairly quickly. These people are said to have “rapid progression.” For others, symptoms may stay the same for long periods of time, known as “stable course.” The majority of people with IPF experience “slow progression,” a slow but steady worsening of their disease.

Types of IPF Progression Graph

Some people may also experience serious and sudden attacks of shortness of breath known as acute exacerbations. The cause of these exacerbations in IPF is still unknown and is being investigated.

Acute exacerbations cause a sudden and often permanent worsening of the disease, which leaves the person with less lung function than they had before. More than just “having a bad day,” an exacerbation may lead to hospitalization. Half of them can be fatal. During an acute exacerbation, you may experience a worsening cough and a fever.

What Can Be Done to Help Manage IPF?

Although there is no cure for IPF, there may be things that you and your doctor can do to help manage the disease.

  • Your doctor may prescribe certain treatments that may help to slow the progression of the disease.
  • Your doctor may also recommend oxygen therapy if breathing on your own becomes too difficult.
  • Pulmonary rehabilitation may help to improve your quality of life.
  • A lung transplant may be an option for some patients. Talk with your doctor about whether or not you could be a candidate.
Although IPF can be an overwhelming disease, we are here to help you understand the condition and possible treatment options. Using the information here, you can have an informed and meaningful conversation with your doctor about how IPF impacts the body and possible treatment options.

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496

The word “idiopathic” (id-ee-o-PATH-ick) means that the cause of idiopathic pulmonary fibrosis (IPF) is unknown. However, although the connection is not clear, IPF seems to occur more commonly in people with certain risk factors. There also may be some therapies or medicines that may cause damage to lung tissue that may lead to IPF. Below are some activities, medical conditions, and treatments that have been found to increase the risk of IPF.

Things that May Increase the Risk of IPF

Possible IPF Risk Factors

Smoking

Even a past history of smoking may increase the risk of IPF

Environment

Long periods of exposure to asbestos fibers, silica, or grain dust

Bird and animal droppings

Chronic viral or bacterial infections

The herpes virus is one virus that is suspected of possibly playing a role in IPF

Certain bacteria may also contribute to lung problems such as IPF

Acid reflux disease

Also known as gastroesophageal reflux disease (GERD)

One theory is that the scarring seen in IPF is due to tiny amounts of stomach acids getting into the lungs, but this has not been proven

Family history of IPF

IPF is known to appear in families, which suggests there could be a problem with a gene that is inherited

Certain Treatments

Certain cancer treatments, such as some types of chemotherapy or radiation

Certain Medicine

Certain types of heart medicine and a few specific, less commonly used antibiotics

Researchers are still working hard to find out exactly what causes certain people with these risk factors to develop IPF.

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1032

The symptoms and severity of idiopathic pulmonary fibrosis (IPF) will vary from person to person. However, here are a few signs and symptoms to look for:

Shortness of breath

Also called dyspnea, this condition continues to worsen and many patients will require the use of supplemental oxygen

Chronic, dry, hacking cough

The cough is usually dry and nonproductive (does not contain mucus)

About 80% of people with IPF have a cough that is typically dry

Crackling breath sounds

When listening to an IPF patient’s lungs with a stethoscope, doctors hear what has been described as a Velcro-like tearing sound

Fatigue and weakness

This symptom is common in all forms of interstitial lung disease

Loss of appetite/unexplained weight loss

This symptom is common in all forms of interstitial lung disease

General complaints

Muscle and joint pain, swelling, and dry eyes and mouth, are often reported in IPF

Clubbing of the fingertips

The flesh under the fingernails gets thicker; this causes the nails to curve downward

  • This happens in approximately 50% of people with IPF

  • Believed to be the result of low oxygen levels in the blood

How You Can Help Your Doctor

Symptoms of IPF, such as breathlessness, chest tightness, cough, and fatigue, can be similar to those of other diseases. For example, asthma, chronic obstructive pulmonary disease (COPD), and congestive heart failure (CHF) share some of these same symptoms. What’s more, your primary care doctor may not have seen many (if any) patients with IPF before. In fact, up to 50% of cases of IPF are misdiagnosed.

Help Your Doctor Diagnose IPF SymptomsIf you have some or all of these symptoms, describe them carefully to your doctor. You may also want to consult a lung specialist (a pulmonologist). This type of doctor specializes in the treatment of lung disease. However, not all pulmonologists are experienced with IPF. This is why you may want to try to find one who is familiar with the diagnosis of IPF, the course of IPF, and the latest approaches to managing its symptoms.

With IPF, you may not even experience any symptoms at the early stages. However, most patients find that their lung function grows gradually worse over time. Some patients may experience “acute exacerbations.” This is when you experience a sudden, significant worsening of lung function, with no apparent cause. These can lead to serious consequences such as permanent loss of lung function and hospitalization. (Learn more about exacerbations.)

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169

When it comes to diagnosing IPF and other respiratory conditions, it’s important that your doctor understands your symptoms, possible risk factors, and medical history. The more information you can provide your doctor, the better prepared he or she will be to make an accurate diagnosis. We’ll help you share this information with your doctor by creating a personalized Doctor Discussion Guide. All you need to do is:

  • Step 1: Answer the questions below to pinpoint your symptoms, possible risk factors, and medical history.
  • Step 2: Print or e-mail a copy of your completed guide and bring it to your next appointment to help start a conversation with your doctor.
Please answer question(s):

Question
1 of 5error message


What symptoms are you experiencing? Select all that apply.

Shortness of Breath, IconShortness of Breath, Icon

Shortness of breath with or without activity that worsens over time

Long-Lasting Dry, Hacking Cough, IconLong-Lasting Dry, Hacking Cough, Icon

Dry, hacking cough that lasts a long time

Wet Cough, IconWet Cough, Icon

Wet cough that produces mucus

Fatigue, IconFatigue, Icon

Fatigue or generally not feeling well

Unexplained Weight Loss, IconUnexplained Weight Loss, Icon

Unexplained weight loss

Chest Pain or Tightness, IconChest Pain or Tightness, Icon

Chest pain or tightness

Sleep Apnea, IconSleep Apnea, Icon

Difficulty breathing during sleep (sleep apnea)

Clubbed Fingertips, IconClubbed Fingertips, Icon

Clubbing (or widening) of the fingertips

Chronic Heartburn/Acid Reflux, IconChronic Heartburn/Acid Reflux, Icon

Chronic heartburn/acid reflux (GERD)

None/No, IconNone/No, Icon

None

Other, IconOther, Icon

Other

Next

Question
2 of 5error message


Have you been treated for any of the following conditions (with treatments like inhalers, antibiotics, etc.) but have been unable to find relief from your symptoms?
Select all that apply.













Next

Question
3 of 5error message


Do you currently smoke or have you smoked in the past?

Yes, IconYes, Icon

Yes

None/no, IconNone/no, Icon

No

Next

Question
4 of 5error message


Have you been regularly exposed to any of the following?
Select all that apply.






Next

Question
5 of 5error message


Have any of your family members been diagnosed with a respiratory disease?

Yes, IconYes, Icon

Yes

None/No, IconNone/No, Icon

No

Unsure, IconUnsure, Icon

Not sure

Have any of your family members been diagnosed with:
Select all that apply.







Get Your Guide

Below is a record of your symptoms, risk factors, and medical history based on your answers to a few questions. We’ve also included a list of questions that you may want to ask your doctor. Be sure to print or e-mail this guide and bring it to your next appointment. That way, you’ll have the information you need to begin a more meaningful conversation with your doctor.

My Summary

Response 1

I experience the following symptoms:










Response 2

I have been treated for the following conditions (with treatments like inhalers, antibiotics, etc.) but have been unable to find relief from my symptoms:













Response 3

I currently smoke or have smoked in the past:

Response 4

I have been regularly exposed to pollutants, such as:






Response 5

I have family members who have been diagnosed with a respiratory disease:

I have family members who have been diagnosed with:







Questions for My Doctor

Below is a list of questions that you should be sure to ask your doctor. Please note that this list is not exhaustive. There are other possible questions that you may want to ask your doctor.

Question 1

I’ve been experiencing symptoms for some time now and haven’t found relief from any treatments we’ve tried. Do you think I could be suffering from a condition that I haven’t been treated for?

Question 2

When you listen to my lungs, do you hear a Velcro-like crackle? What does that mean? If not, what sounds—if any—do you hear?

Question 3

Are there other tests we can do to help determine the cause of my symptoms?

Question 4

Will I need to see a specialist? If so, can you refer me?

Get a Copy


Don't forget to print or e-mail your guide and take it
to your next appointment.


E-mail



Download



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685

Diagnosing idiopathic pulmonary fibrosis (IPF) is often a long and difficult process. That’s because IPF is a rare disease that some doctors are not familiar with. Its symptoms—including a persistent cough, shortness of breath, and fatigue—are common and found in many respiratory and other diseases, making it easy to confuse IPF with other conditions. For many, it can take 1 to 2 years to receive a correct diagnosis after symptoms occur.

Receiving an early diagnosis, however, is very important. That’s because IPF is a progressive disease —meaning it worsens over time. When a person receives an early diagnosis, treatments that slow disease progression can be started earlier. The sooner you receive a diagnosis, the sooner you can begin to evaluate your treatment options with your doctor. There are steps you can take to help speed up the diagnostic process.

Preparing for a Possible Diagnosis

Assessing Your Symptoms and Risk Factors

The first step in the diagnostic process is having a clear awareness of your symptoms. Once you are aware of your symptoms, you’ll be better prepared to explain exactly how you’re feeling to your doctor. The more information your doctor has about your symptoms and medical history, the better.

Sometimes it can be difficult to remember all of your symptoms during your appointment. Therefore, it may be helpful to actually record this information beforehand. That way, you can go to your appointment prepared to give your doctor a clear and complete picture of your symptoms.

Finding a Pulmonologist

A pulmonologist is a doctor who specializes in lung diseases. If you think you could be suffering from IPF, you may want to ask your primary care physician if you should be referred to a pulmonologist. Pulmonologists are likely to be familiar with IPF and, therefore, may be able to provide an accurate diagnosis faster.

There are also medical centers that specialize in lung diseases. These medical centers likely have the expertise necessary to diagnose and treat a patient with IPF.

What to Expect During Your Appointment

Diagnostic Tests and Tools

Now that you know how to prepare for your appointment, let’s discuss what you can expect at the appointment itself. Along with a review of your medical history and a physical exam, your doctor will likely use several tests to diagnose IPF. These may include:

High-Resolution Computerized Tomography (HRCT) Scan of the Lungs

Offers a detailed lung image to help your physician identify scar tissue and diagnose or rule out IPF at an early stage.

Surgical Lung Biopsy

If necessary, during a lung biopsy, your doctor will take samples of lung tissue and then examine them under a microscope to help rule out other diseases or diagnose IPF.

Arterial Blood Gas

If necessary, your doctor will perform a blood test that measures the amount of oxygen and other gases present in the blood.

Bronchoscopy with Broncho-alveolar Lavage

If necessary, a doctor uses a thin, flexible tube with a small camera attached to the end to examine the airways in the lungs during a bronchoscopy.

Broncho-alveolar lavage can be performed during a bronchoscopy and allows doctors to collect cells from around the air sacs in your lungs that can be examined.

Chest X-ray

Provides an image of the structures in your chest, including your lungs. May be used to check for scar tissue.

Exercise Test

One type of exercise test is the “6-minute walk test”; during this exercise test, your doctor will have you walk to determine how well your heart and lungs function during physical activity.

Pulse Oximetry

Involves the use of a small sensor placed on your fingertip or earlobe. It uses light to measure the amount of oxygen in your blood.

Lung Function Tests

Measures characteristics when you breathe in and out of a tube.

If you are having symptoms or think you could be suffering from IPF, be sure to make an appointment with your doctor. At the appointment, remember to talk with your doctor about your symptoms and possible diagnostic tests. That way, you can help your doctor understand how you’re feeling and—ultimately—may receive an accurate diagnosis sooner.

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331

If you’ve been diagnosed with IPF, your doctor will likely use tests to track how the disease progresses over time. Knowing how your condition is changing over time can help you and your doctor make informed treatment decisions.

You can find a list below of some of the tests your doctor may use. Note that this is just for your information. Your doctor may not use all of these. They may even employ tests not listed here.

Medical Tests Used to Track the Progression of IPF

High-Resolution Computerized Tomography (HRCT) Scan of the Lungs

Offers a detailed lung image to help your physician see how scarring of the lungs has progressed.

Lung Function Test

Used to measure how much air you can blow out of your lungs. The amount of air will likely decrease as IPF progresses.

Pulse Oximetry

Involves the use of a small sensor placed on your fingertip or earlobe. It uses light to measure the amount of oxygen in your blood.

You can also take part in tracking your IPF by keeping a record of your symptoms over time. Use our Symptom Tracker to document how you’re feeling each day to be shared with your doctor.

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735

The symptoms of idiopathic pulmonary fibrosis (IPF) are not always noticeable or bothersome until the disease has already progressed. As the disease continues to progress, most patients with IPF experience a gradual worsening of lung function. But the course of IPF varies a great deal from one patient to another.

Unlike many other chronic conditions, there are no "stages" of IPF. Everyone experiences it differently. Data suggest that the median life expectancy tends to be just 2-3 years following diagnosis. This is the “median” or middle survival time. This means that some patients may only live a shorter time, while some others may live several years longer. Scientists have yet to determine which factors lead one IPF patient to survive longer than another. Research has shown that there may be different “subtypes” of IPF. It’s possible that patients with a certain IPF subtype may actually live longer than other patients with a different IPF subtype, although this is still being investigated.

About Breathlessness

Most people with IPF experience shortness of breath, which doctors call “dyspnea” (“DISP-knee-ah”). Some patients ignore occasional breathing troubles, believing it’s just a sign of old age or being out of shape. However, as IPF progresses, the damage to the lungs grows more severe, and breathlessness becomes more common. It may occur with minor physical activity—such as getting dressed—or while at rest. With advanced disease, something as simple as brushing your teeth may even lead to breathlessness. Breathlessness has also been reported to lead to other problems, such as trouble with swallowing.

IPF Lung Damage

What Are Exacerbations?

People with IPF may experience complications that may cause an increase in symptoms or a decline in lung function. These complications may also lead to an appearance of new patterns on an HRCT image. (Learn more about tests used to monitor progression of IPF.) When this happens, it is known as an “acute exacerbation” (“ex-zas-sir-BAY-shun”) of IPF. The cause of exacerbations in IPF is still unknown and is being investigated.

What Can Happen During an Exacerbation?

  • You may feel your breathing become more difficult than usual, and this feeling may not get better over time (it may even last up to 30 days)

  • You may experience a worsening cough

  • You may develop fever or flu-like symptoms

  • In severe episodes, you may have trouble breathing on your own

Acute exacerbations of IPF are a very serious concern because they can lead to a rapid decline in lung function. After an exacerbation, lung function does not return to where it was before. More than just “having a bad day,” an exacerbation can lead to hospitalization. It may ultimately lessen your independence and prevent you from continuing to take part in certain activities. As of now, there are no clear data to suggest that therapies used to treat acute exacerbations of IPF have any benefit while they are occurring, although FDA-approved treatment can reduce their occurrence.

Who Gets Exacerbations?

IPF Patients Who Get Exacerbations It’s important to note that not all people with IPF will experience acute exacerbations. One study found that only about 10% of people with IPF had exacerbations over a 2-year period. Also, the cause of exacerbations is still unknown. With this in mind, the best approach is to work with your doctor. He or she may help you find the right balance between taking part in those events that matter to you and your family and being appropriately cautious about situations that may pose a risk to someone with a serious lung disease like IPF.

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1474

Idiopathic pulmonary fibrosis (IPF) is a rare and serious irreversible lung disease. 'Idiopathic' means that the cause of the disease is unknown. 'Pulmonary' means it's a disease that impacts the lungs. 'Fibrosis' means scarring. In all, 'IPF' means an unknown factor is causing scarring of the lungs.

Idiopathic pulmonary fibrosis (IPF)
[ID-ee-oh-PATH-ik PUHL-mon-air-ee fi-BRO-sis]


Over time, this scarring makes it difficult for the lungs to expand as you breathe. This is what's known as a restrictive lung disease. However, the non-specific symptoms of IPF may lead to misdiagnoses of obstructive lung diseases like chronic obstructive pulmonary disease (COPD) and asthma. Unlike IPF and other restrictive lung diseases where the lungs can't fully expand as you inhale, obstructive lung diseases occur when there is lung impairment due to a blockage or narrowing of the airways.

If you are wondering if you may have IPF, you may want to bring this information with you when you see your doctor.

IPF by the Numbers

Up to
132,000
people in the US are currently affected by IPF
40K
There are 30,000 to
40,000 new cases each
year
50
Usually affects
people at least 50 years
old
1/2
Over 50% of cases
are misdiagnosed
at first

Diagnosing and Managing IPF

Diagnosing IPF can be a long and difficult process. In fact, over 50 percent of IPF cases are misdiagnosed at first. That's because the symptoms of IPF are easily confused for other conditions like COPD, cardiac disease, or simply aging.

However, early diagnosis of IPF is important because it is a progressive disease. That means the condition will worsen over time—sometimes very quickly. While there is no cure for IPF, there are treatments available that may help slow the progression of the disease. The sooner you receive a diagnosis, the sooner you can work with your doctor to assess your treatment options.

Your doctor will likely use several tests and tools to make a diagnosis, including special chest x-rays known as high-resolution computed tomography (HRCT) and lung function tests. A lung biopsy (obtaining lung tissue) is sometimes necessary. Your doctor may refer you to a pulmonologist. A pulmonologist is a doctor that specializes in treating lung diseases, and likely has the expertise required to diagnose and treat IPF.

Need help locating a pulmonologist near you?

Finding Support

IPF can be an overwhelming and isolating disease. But it's important to remember that you're not alone. It may be helpful for you to connect with other people who are living with IPF. Plus, we're here to offer the information and continued support you need to manage life with IPF. All you need to do is sign up to receive IPF information, tools, and more right in your inbox.