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Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason. Over time, the scarring gets worst and it becomes hard to take in a deep breath and the lungs cannot take in enough oxygen.

IPF is a form of interstitial lung disease, primarily involving the interstitium (the tissue and space around the air sacs of the lungs), and not directly affecting the airways or blood vessels. There are many other kinds of interstitial lung disease that can also cause inflammation and/or fibrosis, and these are treated differently. It is important to work with your doctor to determine if you have IPF or another form of interstitial lung disease.

Causes

The cause of idiopathic pulmonary fibrosis is not completely understood. No one knows what causes idiopathic pulmonary fibrosis or why some people get it. “Idiopathic” means of unknown cause. Common risk factors for IPF include:

  • Genetics (family history): Up to 20% of people with IPF have another family member with an interstitial lung disease. If more than one member of your family has IPF, the disease is called familial pulmonary fibrosis.
  • Cigarette smoking: Approximately 75% of people with IPF are current or previous cigarette smokers.
  • Acid reflux (gastroesophageal reflux disease [GERD]): Approximately 75% of people with IPF have symptoms of acid reflux or heartburn.
  • Male sex: Approximately 75% of patients with IPF are male.
  • Age: Almost all patients with IPF are over the age of 50 years

Symptoms

The two main symptoms of IPF are:

  • Breathlessness (also known as shortness of breath or dyspnea): Usually the breathlessness of IPF first appears during exercise. Breathlessness can affect day-to-day activities such as showering, climbing stairs, getting dressed and eating. As scarring in the lungs gets worse, breathlessness may prevent all activities.
  • Chronic cough: About 85% of people with IPF have a chronic cough that has lasts longer than 8 weeks. This is often a dry cough, but some people may also cough up sputum or phlegm.

Other symptoms may include:

  • chest pain or tightness
  • unexplained weight loss
  • loss of appetite
  • tiredness and loss of energy
  • change of finger shape called “clubbing”

Diagnosis

How is idiopathic pulmonary fibrosis (IPF) diagnosed?

Idiopathic pulmonary fibrosis (IPF) can be difficult to diagnose since many lung diseases can cause breathlessness and cough, and some of these other diseases can have similar symptoms and X-ray results. You may have to see more than one specialist, including a respirologist, to help with the diagnosis.

  • Your doctor will ask you questions about your home and work environments, your smoking history and your medical history. This will help them determine whether there were any environmental, occupational, genetic, or other factors that could be linked to the development of pulmonary fibrosis.
  • Your doctor will do a physical examination of your lungs and will also examine other parts of your body including your heart, joints, fingernails, skin and muscles.

Although no single test can diagnose IPF, your doctor may recommend a number of different tests including:

  • Pulmonary function test (PFT): A PFT, or breathing test, is a simple test to measure 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 test helps determine your physical fitness, as well as the amount of oxygen in your blood at rest and with physical activity.
  • Chest x-ray: Chest X-rays are simple images that can screen for interstitial lung disease and can sometimes be useful to help monitor progression.
  • Blood tests: A number of blood tests can be requested to help identify other causes of interstitial lung disease.
  • Computed tomography (CT scan): These radiology images will determine whether there is scarring in your lungs and will help identify the subtype of interstitial lung disease.
  • Bronchoscopy: Bronchoscopy is a procedure that involves inserting a small flexible tube, called bronchoscope, through your mouth or nose and into your lungs. This is not necessary for diagnosis but some patients with possible IPF undergo this procedure to help identify the presence of infection or to suggest other subtypes of interstitial lung disease.
  • Surgical lung biopsy: Patients with an unclear diagnosis will sometimes undergo a surgical lung biopsy performed by a surgeon under general anesthetic. This test involves small incisions in the side of the chest, which allows removal of lung tissue and its examination under a microscope.

Treatment

There is no cure for IPF and there are currently no procedures or medications that can remove the scarring from the lungs. Treatments are used to slow progression of the lung scarring, and may not necessarily lessen the symptoms of cough and breathlessness. Pirfenidone (Esbriet) and nintedanib have both been shown to slow the progression of IPF; however, some patients cannot take these medications due to their side effects. These medications are not approved for use in other interstitial lung diseases.

Some additional medications can be used to improve the symptoms of IPF, such as shortness of breath and cough. This includes treatment of gastroesophageal reflux with anti-acid therapy and treatment of shortness of breath with opioids (a class of medications that includes morphine). Other treatments include supplemental oxygen, pulmonary rehabilitation (exercise training, education and support for people with chronic lung disease), and lung transplant.


Understand your IPF

It is important to work with your family doctor or respirologist (lung specialist) to find the best treatment for you. Not all medications will work for everyone. Your doctor knows your medical history and will be able to determine the best treatment for you.

The following can help you prepare for your appointment with your doctor:

  • Prepare a written list of the questions you want to ask. Put the most important questions at the top of your list.
  • Take notes as the doctor is answering your questions. If you don’t understand the doctor’s answers, ask for more details.
  • Show the doctor a written list of all your symptoms. Be honest.
  • Bring along all of your medications (including herbal remedies).
  • If you’re not sure you’re taking your medications correctly, ask your doctor to review it with you.
  • Bring a friend or relative to your appointment to help you remember details and take notes for you.
  • Ask your doctor about programs in your community that help people with IPF and other lung diseases. Ask about joining a pulmonary rehabilitation program.
  • Ask your doctor about a local support group for IPF. Obtaining psychosocial support, education and learning coping skills through patient support groups can be very valuable for the patients and their caregivers.
  • Discuss the course of your disease with your doctor at follow-up visits and ask your doctor to help you develop goals for your care.

Your health-care team can help you learn more about IPF and how to cope with it. Local home-care services can be very important in providing you with essential services in your home by liaising with you doctors when the need arises. People on your IPF health-care team may include:

  • doctor(s), including your family doctor and a respirologist (lung specialist)
  • nurse
  • certified respiratory educator, a health professional with special training in lung disease
  • pharmacist
  • respiratory therapist, nurse, or cardiopulmonary physiotherapist who leads your pulmonary rehabilitation program
  • certified respiratory educator from the Lung Association’s free helpline (call 1-866-717-2673)
  • occupational therapists through your doctor’s clinic, pulmonary rehabilitation program or Homecare who can provide you with suggestions on how to live better with breathlessness.

Lifestyle changes

IPF is an irreversible, progressive disease. So it is important to learn good coping skills and educate yourself about the disease as much as possible.

  • Quitting smoking is the most important thing you can do to help slow down the progression of any lung disease.
  • Get a flu shot each year.
  • Ask your doctor about getting a pneumonia shot.
  • Keep away from people with chest infections and colds.
  • Wash your hands properly and often.
  • Stay as fit as you can.

Oxygen

The scarring in the lungs can prevent oxygen from being absorbed into your body. Low oxygen levels can make you feel short of breath and tired. If your blood oxygen levels are too low, your doctor may prescribe supplemental oxygen (home oxygen). This helps to reduce breathlessness and can help you be more active. Some people with IPF may need oxygen therapy all the time, while others may only need it during exercise or sleep.

If your doctor has prescribed supplemental oxygen, it is important to use it as prescribed. Many patients fear that they will become “addicted” to supplemental oxygen. Supplemental oxygen is not addictive and your body needs a proper amount to maintain normal body functions. People who take supplemental oxygen must continue taking their other medications.

Not everyone who has IPF needs to be on oxygen. Generally, oxygen therapy can help only people with very low blood-oxygen levels (hypoxemia). To find out if you need supplemental oxygen, speak to your doctor or certified respiratory educator.


Medications

There are no medications that cure IPF or decrease the amount of scarring in the lungs. Some medications help slow the progression of pulmonary fibrosis.
It is very difficult to find medications that will work for everyone with IPF. Medications for IPF have side effects in some patients, so it is important to talk to your doctor about the possible risks.

As with any medication for any condition, you should talk to your doctor about different treatment options and ask about medication side effects.

Pirfenidone

Pirfenidone is an anti-scarring (anti-fibrotic) medication that slows the progression of IPF. Some patients taking pirfenidone have side effects, most commonly stomach upset and skin rash, particularly with exposure to sun. Pirfenidone has been approved by Health Canada for the treatment of mild to moderate IPF.

Nintedanib
Nintedanib is an anti-scarring (anti-fibrotic) medication that slows progression of IPF. Some patients taking nintedanib have side effects, most commonly including diarrhea.

Corticosteroid pills
Corticosteroid pills (for example, Prednisone©) can reduce inflammation in your lungs by suppressing your immune system. Corticosteroids are only used in patients with IPF who have an acute exacerbation of their lung fibrosis, and can be harmful in patients with IPF that have scarring that is stable or slowly worsening.

N-Acetylcysteine (oral or aerosolized)
N-acetylcysteine (NAC, mucomyst) is an antioxidant that has frequently been used in patients with IPF. A large clinical trial published in May 2014 showed that NAC does not slow progression of IPF.

Other medications
Other medications have been studied and previously used in IPF, including azathioprine, cyclophosphamide, and others. These medications are not recommended in IPF as they have no proven benefit in IPF and are associated with significant side effects.


Pulmonary rehabilitation

Pulmonary rehabilitation is an exercise and educational program designed just for patients with lung disease. Pulmonary rehabilitation can help in teaching patients with IPF how to breathe more efficiently and to perform their activities of daily living with less breathlessness.

Most pulmonary rehabilitation programs are designed for people with COPD (emphysema and chronic bronchitis), although the majority of pulmonary rehabilitation programs in Canada accept and welcome patients with IPF.

Read more about pulmonary rehabilitation and find a program near you.


Lung transplant

Lung transplant is not right for everyone, but it may be a treatment option for some people. It is important to work with your doctor to see if lung transplant is an option for you.
If a person qualifies for a lung transplant, they are put on a waitlist for organ donation. The wait can be long and the surgery can be risky. People who get transplants must take several anti-rejection medications (immune-suppressants) for the rest of their lives.

Managing your IPF

There are things you can do to help you manage your IPF:

Work with your health-care team to manage and understand your IPF

It is important to work with your family doctor or respirologist (lung specialist) to find the best treatment for you. Not all medications will work for everyone. Your doctor knows your medical history and will be able to determine if medication is an option for you.
Here are some things to talk to your health-care team about:

  • Prepare a written list of the questions you want to ask. Put the most important questions at the top of your list.
  • Take notes as the doctor is answering your questions. If you don’t understand the doctor’s answers, ask for more details.
  • Show the doctor a written list of all your symptoms. Be honest.
  • Bring along all your medications (including herbal remedies).
  • If you’re not sure you’re taking your medications correctly, ask your doctor to review it with you.
  • Bring a friend or relative to your appointment. He/she can help you remember details and also take notes for you.
  • Ask your doctor about programs in your community that help people with IPF and other lung diseases. Ask about joining a pulmonary rehabilitation program.

Your health-care team can help you learn more about IPF and how to cope with it. People on your IPF health-care team may include:

  • Doctor(s), you may have a family doctor and a respirologist (lung specialist)
  • A nurse
  • A certified respiratory educator, a health professional with special training in lung disease
  • A pharmacist
  • A respiratory therapist, nurse, or cardiopulmonary physiotherapist who leads your pulmonary rehabilitation program
  • A certified respiratory educator from the Lung Association’s free helpline (call 1-866-717-2673)

Make changes to your lifestyle to stay healthy, and fight germs to prevent infections

  • Quitting smoking is the number one thing you can do to help slow down the progression of any lung disease.
  • Get a flu shot each year.
  • Ask your doctor about getting a pneumonia shot.
  • Keep away from people with chest infections and colds.
  • Wash your hands properly.
  • Stay as fit as you can.

Additional Resources


Researchers are conducting clinical trials in Canada and around the world. Some patients enroll in a clinical trial that tests new treatments of IPF. While the long-term goal of IPF research is to prevent and cure the disease, current studies also attempt to slow disease progression, and to increase the life expectancy of patients with IPF.

Please visit www.clinicaltrials.gov to identify ongoing IPF clinical trials.

For information on how to set up an IPF education and support group, visit the Canadian Pulmonary Fibrosis Foundation website where you can download a toolkit.