Idiopathic Pulmonary Fibrosis (IPF)

Medically Reviewed by Zilpah Sheikh, MD on November 07, 2023
11 min read

Idiopathic pulmonary fibrosis (IPF) is a serious lung disease. When you breathe in, oxygen moves through tiny air sacs in your lungs and into your bloodstream. From there, it travels to your organs. IPF causes scar tissue to grow inside your lungs and makes it hard to breathe. It gets worse over time.

About 48,000 people get IPF each year in the United States.

IPF scar tissue is thick, like the scars you get on your skin after a cut. It slows oxygen flow from your lungs to your blood, which can keep your body from working as it should.

There’s no cure for IPF. For most people, symptoms don’t get better, but treatments can slow the damage to your lungs. Everyone’s outlook is different. Some people will get worse quickly, while others can live 10 years or more after diagnosis. There are treatments to help you breathe easier and manage your symptoms. You may be able to have a lung transplant.

You can have idiopathic pulmonary fibrosis for a long time without noticing any symptoms. 

What does IPF feel like? Your lungs each contain about 300 million tiny air sacs called alveoli that fill with oxygen every time you breathe. In healthy lungs, the walls of these air sacs are very thin, so that oxygen and carbon dioxide can easily pass through them.

When you have IPF, scar tissue forms on the air sac walls and in the spaces around them, making them thick and hard. This scarring makes it harder for air to pass in and out of the sacs. That's why you feel out of breath.

If you have IPF, you may have rapid, shallow breathing or a dry, hacking cough that doesn't go away. When a doctor listens to your breath sounds through a stethoscope, they may hear a crackling noise in your lungs. 

After many years, the scarring in your lungs gets worse, and you might have:

Other symptoms of IPF include:

  • Feeling more tired than usual
  • Joint and muscle aches
  • Weight loss without trying
  • Clubbing, when the tips of your fingers and toes get wider

Doctors don't know what exactly causes IPF.  "Idiopathic" means unexplained, and that's the term doctors use when they can't pinpoint a cause for your condition.

Researchers think certain things you breathed in are linked to IPF, such as wood dust or asbestos. They believe that the scarring is caused by something in the body that attacks the lungs over and over, but they don't know what or why.

IPF can run in families. Some people with the disease have one or more relatives who have it. In these cases, it's known as familial IPF.

Cigarette smoking is linked to IPF. So are some infections, such as the flu, Epstein-Barr virus (which causes mononucleosis), hepatitis, and herpes. These things may increase your risk of the disease, although they are not direct causes.

People with IPF may also have gastroesophageal reflux disease (GERD), also known as acid reflux. Scientists don't know if one disease causes the other, but they suspect that people with GERD may breathe tiny drops of stomach acid into their lungs, which can cause damage over time.

IPF is hard to tell apart from other lung diseases because it shares many of the same signs. It may take time and a lot of doctor visits to get the right diagnosis. If you have trouble breathing that doesn’t get better, you’ll probably need to see a pulmonologist, a doctor who treats lung problems.

The doctor will use a stethoscope to listen to your lungs. They might ask questions like:

  • How long have you been feeling this way?
  • Have you ever smoked or used drugs?
  • Do you work with chemicals at your job or home? Which ones?
  • Has anyone in your family been diagnosed with IPF?
  • Do you have any other medical conditions?
  • Have you ever been told you had the Epstein-Barr virus, influenza A, hepatitis C, or HIV?


Your doctor may ask you to get tests that look for damage in your lungs. They can also help rule out other diseases.

Some you can take in your doctor's office without any special preparation, but for others you'll need to go to a lab or hospital.

Chest scans. An X-ray lets your doctor see the structures inside your body. It may show shadows on your lungs that suggest scar tissue.

You may also need an HRCT scan (high-resolution computed tomography). It's a sharper and more detailed type of X-ray that can spot signs of IPF at an earlier stage.

Breathing test. Your doctor may use a device called a spirometer to measure how well your lungs are working.

You take a deep breath in and then blow as hard as you can into a tube connected to the device. You'll wear a clip on your nose so you can only breathe in and out of your mouth.

Pulse oximetry. Your doctor clips a small sensor to the tip of your finger or ear. It sends a painless beam of light through your skin to check the oxygen level in your arteries.

Blood test. It's used to check your oxygen and carbon dioxide levels.

Your doctor takes blood from an artery on the inside of your wrist, arm, or groin. It may be slightly more painful than a regular blood test, which takes a sample from your veins.

You may feel some discomfort. You'll need to sit calmly and keep pressure on the spot for a few minutes afterward to keep it from bleeding.

Skin test. Tuberculosis causes symptoms that are like IPF, so you may need a test to rule out this disease. Your doctor uses a tiny needle to inject a substance under the top layer of skin on your arm. This will form a small bubble, like a blister.

You'll need to see your doctor or lab technician 48 to 72 hours later to see if there's a reaction, which will look like a red, swollen bump.

Exercise test. It measures how well your lungs push oxygen through your bloodstream while you're moving around. You may have to walk on a treadmill or pedal a stationary bike while hooked up to devices that measure your heart rate, blood pressure, and how much oxygen is in your blood.

Lung biopsy. The best way for your doctor to diagnose IPF is to take small samples of tissue from your lungs and check them under a microscope for signs of scarring or other disease.

There are different ways to do it. Your doctor may put a needle through your chest, or they may put a long, thin tube through your mouth and down your throat.

For some biopsies, you'll only need "local anesthesia," which is medicine that numbs an area on your body. For others you'll need drugs that make you sleep while the procedure is done.

You may be asked to stop eating for 8 hours before the biopsy. Make sure to find out if there are any other ways you need to prepare.

Other lung tests. You might need to go to a hospital to get other exams done. For example, you could get a video-assisted thoracoscopy or bronchoscopy. Your doctor puts a tiny tube with a camera on the end through a cut in your chest or into your nose or mouth.

For a test called a bronchoalveolar lavage, your doctor injects salt water into your lungs to collect tissue samples.

Another option is a thoracotomy. Your doctor removes small pieces of lung tissue through a cut between your ribs.

You'll get medicine that puts you to sleep during these tests. Talk to your doctor about how you should prepare and what you should expect afterward.

You might want to ask:

  • How do you know I have IPF?
  • Do I need more tests?
  • Do I need to see any other doctors?
  • Which treatments might work best for me?
  • How will they make me feel?
  • Will anything help me breathe better right away?
  • Are there any clinical trials that would be good for me?
  • How often should I see you?
  • Will I need a lung transplant?
  • Will my children get IPF?

Treatments for idiopathic pulmonary fibrosis won't cure the disease, but they can make it easier for you to breathe. Some may keep your lungs from getting worse quickly. Your doctor may suggest:

  • Medicine. Two drugs, nintedanib (Ofev) and pirfenidone (Esbriet), are approved to treat IPF. They block a process in your lungs that leads to scarring. They may keep your IPF from getting worse and help you breathe better.  You might take other medications to fight the inflammation that makes you feel sick or causes scarring. Your doctor may suggest corticosteroids like prednisone or drugs that slow down your immune system, like azathioprine (Imuran), cyclophosphamide (Cytoxan), and mycophenolate mofetil (CellCept). Another drug, N-acetylcysteine, or NAC (Mucomyst), is an oral or spray antioxidant widely used to treat IPF. Some studies have questioned how well it works. But a large review of results in 2019 concluded the drug was safe and may help, especially when used with other treatments.
  • Oxygen therapy. It helps you feel less short of breath so you can stay active. You breathe oxygen through a mask or prongs that go in your nose.  Whether you need to wear oxygen depends on how serious your condition is. Some people with IPF need it only when they sleep or exercise. Others need it 24 hours a day.
  • Pulmonary rehabilitation. You work with a team of doctors, nurses, and therapists on ways to manage your symptoms. You might focus on exercise, healthy eating, relaxation, stress relief, and ways to save your energy. You may visit the hospital for a rehab program or do one at home.

If your IPF scarring is severe, your doctor may suggest a lung transplant. Most people only think about doing this if other treatments aren't working.

Most people who get lung transplants are younger than 65. But if you're older and have no other serious medical problems, it may be an option for you.

It's a major operation. There aren't many lungs available from donors, so you'll go on a list to receive one. While you wait, avoid smoking, eat a healthy diet, do your breathing exercises, and stay as active as possible.

Once you get word that a donor has been found, you may have to go to the hospital quickly for your surgery, so make sure your doctor can reach you at all times.

You won't be awake during your surgery. Doctors put tubes into your mouth and windpipe that help you breathe while it's going on.

During the operation, your surgeon removes your lung and the blood vessels that connect it to your heart. They then attach your new lung to these blood vessels.

If you're getting two new lungs, you'll need a heart-lung bypass machine to pump oxygen into your blood during the surgery.

A single lung transplant takes 4-8 hours, and a double transplant takes 6-12 hours.

After surgery, you'll recover in the hospital for up to 3 weeks. You'll need to cough to clear fluid from your lungs. You'll also do breathing exercises to learn to take slow, deep breaths.

For 3 months after your surgery, your doctor will test your blood and lungs to make sure your body doesn't reject your new lung or you don't get any infections. You'll take medicines for the rest of your life to help prevent this from happening.

It can take weeks or months to recover. If you're thinking about getting a transplant, you'll need a lot of emotional support. Ask your doctor about support groups that have people facing the same concerns as you. Also find out about educational workshops that can explain what to expect before and after surgery.

Idiopathic pulmonary fibrosis is a serious disease. It will have a big effect on your life and your loved ones. To stay as healthy as possible, follow your treatment plan and see your doctor regularly to make sure your treatment is working.

Other things you can do to feel better include:

  • Eat healthy. A well-rounded diet of fruits, vegetables, whole grains, low-fat or fat-free dairy, and lean protein is good for your body in general. Eating smaller meals more often will also give your lungs more room to breathe.
  • Exercise. Take a daily walk or bike ride. It can strengthen your lungs and reduce stress. If it’s hard to breathe when you’re active, ask your doctor about using oxygen during your workouts.
  • Quit smoking. Cigarettes and other tobacco products damage your lungs and make breathing problems worse. Your doctor can suggest programs to help you quit.
  • Get all your vaccines and boosters. Vaccines can protect you from infections like COVID-19, the flu, whooping cough, or pneumonia, which can harm your lungs. You need a flu shot every year. You’ll need two vaccines to help protect against a serious type of pneumonia. You'll also need to stay up to date with COVID-19 boosters. Ask your doctor about when you should get these vaccines. Try to stay away from people who have colds.
  • Find ways to relax. Low-key things that you enjoy are great ways to fight stress. Try reading, drawing, or meditating.

IPF life expectancy. How long you'll live with IPF depends on many things, including how old you were when you got your diagnosis, what stage your IPF is in, and how it's treated. Doctors don't know the average life expectancy. You may see online sources that say you'll live 3 to 5 years after diagnosis, but those numbers are outdated and don't take into treatment options you have now. 

It can be hard to manage an illness like IPF. Remember that it’s OK to ask a doctor, counselor, friend, or family member for help with any stress, sadness, or anger you may feel. Support groups are good places to talk to other people who are living with IPF or a similar condition. They can give you and your family advice and understanding.

The scar tissue in your lungs makes it hard for your body to get oxygen, which puts a strain on your other organs. IPF can raise your odds of getting other conditions, including:

  • High blood pressure in the lungs, called pulmonary hypertension
  • Heart attack
  • Stroke
  • Blood clots in your lungs
  • Lung cancer
  • Lung infections

Ask your doctor what you can do to lower your chances of having these problems. There may be treatments that can help with these conditions, too.

Everyone with IPF is different. For some, the disease gets worse quickly. For others, it can be a slow process in which their lungs stay the same for a long time. Talk to your doctor about your condition and what you can do to manage it.

Average survival is 3 to 5 years, but some people live much longer. A lung transplant can also change your life expectancy.

To learn more about IPF or find a support group in your area, visit the website of the Pulmonary Fibrosis Foundation.