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Idiopathic Pulmonary Fibrosis (IPF)

About idiopathic pulmonary fibrosis (IPF)

IPF is a chronic lung disease. This means that is a long-lasting disease that needs treatment. The name idiopathic pulmonary fibrosis tells you a little bit about the disease:

  • Idiopathic means the cause is not known.
  • Pulmonary means it affects the lung.
  • Fibrosis – the scarring caused by IPF is called fibrosis.

There are other words that you might hear when talking to people about IPF.

  • Interstitial – the scarring occurs in the tissue and spaces between the tiny air sacs and blood vessels in the lung. This area is called the lung’s interstitium.

There are many other kinds of interstitial lung diseases and they are treated differently than IPF. It is important to work with your doctor to find out if you have IPF or another form of interstitial lung disease.


What does IPF do to the lungs?

The lung contains airways, air sacs and blood vessels. There is tissue in the small spaces between the airs sacs and tiny blood vessels known as capillaries. IPF is a disease where this tissue becomes inflamed or irritated and swollen.

Over time, this inflammation causes scarring. As the scarring gets worse the lungs become stiff. This makes it harder to take in a deep breath. It also makes it harder for the lungs send oxygen into the blood.

Click here to see a video about IPF.


What are the symptoms of IPF?

The two main symptoms of IPF are breathlessness and chronic cough.

  • Breathlessness is sometimes called dyspnea or shortness of breath. Most people notice it first during exercise or physical activities, like walking up hills. Breathlessness gets worse as the lung’s scarring gets worse. It can affect day-to-day activities such as showering, climbing stairs, getting dressed and eating.
  • Chronic cough bothers most people with IPF. It is called chronic because it lasts longer than 8 weeks. Most of the time the cough is dry, but some people may cough up sputum or phlegm.

People with IPF sometimes have other symptoms, such as:

  • Tiredness and loss of energy
  • Chest pain or tightness
  • Unexplained weight loss
  • Loss of appetite
  • Change of finger shape called "clubbing"

How is IPF diagnosed?

Idiopathic pulmonary fibrosis can be difficult to diagnose because many lung diseases cause breathlessness and cough. Some lung diseases also have similar chest x-rays. You may have to see specialists, including a respirologist (lung specialist) before you get a diagnosis. Here are some of the things that happen on the way to diagnosis:

  • You will be asked questions to help find out if there are factors linked to pulmonary fibrosis. These questions might be about your
    • home and work environments;
    • smoking history;
    • medical history.
  • You will receive a physical examination of your lungs. You will alo have an examination of other parts of your body including your heart, joints, fingernails, skin and muscles.

There is no single test can diagnose IPF. That means you may need several different tests including:

  • Pulmonary function test (PFT): This is a breathing test that measures how much air you can blow in and out of your lungs and how well your lungs can absorb oxygen.
  • Six-minute walk test: This walking test helps measure your physical fitness, and measures amount of oxygen in your blood at rest and during the walk.
  • Chest x-ray: This is a simple image of your lungs that can show interstitial lung disease. Sometimes it can be useful to help monitor progression.
  • Blood tests: Different blood tests can help identify causes of interstitial lung disease.
  • Computed tomography (CT scan): This special type of x-ray helps show scarring in your lungs. The type of scarring can help identify the subtype of interstitial lung disease.
  • Bronchoscopy: This test includes sending a small flexible tube, called bronchoscope, through your mouth or nose and into your lungs. The test does not diagnose IPF but can help find infection causing symptoms. It can also help find other types of interstitial lung disease.
  • Surgical lung biopsy: This test is performed by a surgeon. The test requires a small cut in the side of the chest so that small pieces of lung tissue can be removed. The tissue is examined under microscope to help make diagnosis clear.

What makes getting IPF more likely?

No one knows what causes idiopathic pulmonary fibrosis or why some people get it.

We do know some common risk factors for IPF. These include:

  • Age: Almost all patients with IPF are over the age of 50 years
  • Cigarette smoking: Approximately 75% of people with IPF currently smoke tobacco or did smoke in the past.
  • Acid reflux (gastroesophageal reflux disease [GERD]): About 75% of people with IPF have symptoms of acid reflux or heartburn.
  • Sex: About 75% of patients with IPF are male.
  • Genetics (family history): Up to 20% of people with IPF have another family member with an interstitial lung disease.

While there are things on this list that cannot be changed, some can be changed to reduce the risk.

  • If you smoke, quit smoking. The sooner the better. Stay away from secondhand smoke. Don’t allow smoking in your home or other closed spaces, e.g., car.
  • If you have heartburn, speak to your health care team about what treatments you should use.