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Idiopathic Pulmonary Fibrosis is a progressive stiffening of the lungs. Now the lung is meant to expand and contract, like a balloon. And it takes in oxygen to help diffuse into the blood stream to get to our tissues. And it's made up of a tissue call fibrous cells and they give the lung its structure. However, with Idiopathic Pulmonary Fibrosis, there's an over production of these fibrous cells and the lung becomes stiff and can't expand and take in as much oxygen as it normally does.


So the exact causes of Idiopathic Pulmonary Fibrosis are unknown. However we do have a fairly good idea of what some possible causes could be and they come from chronic trauma to the lungs that causes damage. Some fo these things could be smoking, which causes chronic irritation to the lungs. Another thing could be dust from a worksite. Or even the effects of heartburn which causes acid to come up from the esophagus and go back into the lungs.


The usual symptoms of IPF would be anything that affects the lungs as you can imagine. If you can't breathe very well, you'll be short of breath. Now in the beginning of the disease, you may be able to walk several blocks without any problem. But as it progresses, it's hard to walk one block without having to sit down because you're so short of breath. Another very common symptom is a chronic cough. It may or may not be productive.


The treatment for IPF used to not be very good. We tried anti-inflammatory medications and steroids, but they never really worked. Because, as we know now, the disease is not about inflammation but more about the proliferation of these fibrous cells. However, there are 2-new medications that actually target the fibrous cells and these two medications are Esbriet and Ofev. What they do is that they block the proliferation of these fibrous cells so it really slows the progression of the disease.


An exacerbation is something that causes a worsening of the symptoms. And an exacerbation would come from something like an infection. The infections in someone with Idiopathic Pulmonary Fibrosis can be very dangerous and so we treat them right away with antibiotics and steroids to stop the inflammation from the infection, not from the disease itself. Other things would be supplemental oxygen so you see people wearing oxygen tanks. This can help as well.

Living with IPF

Lifestyle changes that people should consider if they do have Idiopathic Pulmonary Fibrosis would be: one, stopping smoking, two, treating their heartburn, and three, doing something called pulmonary rehab which are exercises designed to improve your lung function. Although in the past, we never had good treatment for Idiopathic Pulmonary Fibrosis, now with these new medications we can really preserve the quality of life. So make sure to take those medications regularly, do your pulmonary rehab, and always consult regularly with your physician.

What Is Idiopathic Pulmonary Fibrosis?

To begin, fibrosis is the buildup of scar tissue. Pulmonary fibrosis is the formation of such scar tissue in the lungs, between the air sacs. Idiopathic just means that your doctors aren’t sure what caused it. So, to put it all together, idiopathic pulmonary fibrosis (IPF) is when the tissue in the lungs becomes damaged or scarred and the cause may be unknown. This thickened, sometimes stiff scar tissue can make it difficult for your lungs to work properly, leading to reduced oxygen levels in your bloodstream.

What Are the Symptoms?

Because your lungs aren’t able to work as efficiently, you’ll likely feel a shortness of breath. This tends to worsen as the disease progresses. You may also experience other symptoms, including:

  • fatigue
  • dry, hacking cough
  • muscle or joint pain
  • sudden or unexplained weight loss
  • clubbing, or when the fingertips and nails become enlarged or rounded

How Did I Get It?

Because it’s idiopathic, your doctors aren’t sure how you developed IPF. Many doctors believe that something either inside or outside of the lungs attacks the lung tissue. The following are just some of the possible causes of these attacks.

Radiation Therapy

Sometimes damage can occur in the lungs of people who received radiation treatments for breast or lung cancer.

Certain Medications

A handful of medications have been shown to increase the risk for pulmonary fibrosis. These include:

Environmental Toxins

Exposure to certain types of pollutants, especially over a long period of time, may contribute to IPF. Some of these pollutants are:

  • asbestos
  • silica and metal dust
  • bird droppings and animal dander
  • dust from grains
  • regular household dust and the various bacteria and other matter it contains
  • cigarette smoke
  • fumes of various types

Medical Conditions

Medical conditions that can damage the lungs increase the risk for IPF, such as:

  • tuberculosis
  • gastroesophageal reflux disease (GERD)
  • rheumatoid arthritis
  • scleroderma
  • pneumonia
  • lupus
  • several viruses, including HIV, hepatitis C, mononucleosis, herpes virus 6, and influenza A


For some, IPF may run in the family. For this reason, it’s thought that genetics may play a role, at least for some people who develop pulmonary fibrosis.

What Can I Expect?

IPF is a serious and progressive disease, with no known cure. You should talk to your doctor so that you can understand your risk and be prepared for complications like:

  • pulmonary hypertension (high blood pressure in the lungs)
  • stroke
  • pulmonary embolism (blood clots in the lungs)
  • heart attack
  • respiratory failure
  • heart failure in the right ventricle, because of how hard the heart is working to pump blood through the damaged arteries in the lungs

What Treatments Are Available?

Unfortunately, few treatment options exist. And, out of the available treatment, they vary in effectiveness. Sometimes, your doctor may recommend a combination of treatments, such as:

  • medications like corticosteroids and antibiotics
  • oxygen therapy
  • pulmonary rehabilitation
  • flu and/or pneumonia vaccines
  • lung transplant, usually as a last resort

Two new treatment options available include pirfenidone and nintedanib. Both medications are believed to decrease the buildup of scar tissue in the lungs and slow the progression of the disease. One trial found that the disease slowed in progression in people who took 150 mg of nintedanib twice a day. 

New and potentially life-saving medications are currently being studied as part of the Idiopathic Pulmonary Fibrosis Network, or IPFNet. Also, the National Heart, Lung, and Blood Institute sponsors clinical trials. 

How Can I Improve My Quality of Life?

To make living with IPF a bit easier, it’s important to keep yourself as healthy as possible. Here are some general tips to keep in mind:

  • Stop smoking, if you do. Quitting can help you cut down on further damage to your lungs.
  • Eat a nutritious diet. You may find that eating becomes more difficult as your breathing gets harder. Eating smaller meals more frequently can make breathing easier.
  • Stay active. Ask your doctor about moderate exercise that can help you stay healthy. You may need to use an oxygen tank while exercising.
  • Get adequate rest.
  • Stay away from places at high altitudes, if possible, and avoid traveling by airplane.

Having IPF may be difficult, but there’s hope. Look for support groups in your area, to find others you can talk to and share tips with. Check with the Coalition for Pulmonary Fibrosis, the Pulmonary Fibrosis Association, and the American Lung Association.