15 Facts About Pulmonary Fibrosis

pulmonary fibrosis

Pulmonary fibrosis is a rare, but serious chronic lung condition that involves scarring between and on the walls of the alveoli in the lungs. As the scarring becomes more severe, the lungs become stiff rather than elastic, and they are no longer able to expand and contract as necessary. This makes it difficult for oxygen to enter the lungs and reach the bloodstream.

Scientists are still in the early stages of learning about and researching pulmonary fibrosis. That being said, we have 15 facts about pulmonary fibrosis to help you learn more about this chronic lung condition.


  1. There are 7 main symptoms of pulmonary fibrosis.
    Symptoms of pulmonary fibrosis are similar to those of other chronic lung diseases. The main symptoms are:
    • Chest pain
    • Dry, hacking, persistent cough
    • Clubbing of the fingers and toes, wherein the ends of the fingers and toes may become larger and rounded (what is called “drumstick” fingers or toes) because of fluid pooling at the ends of the digits
    • Extreme fatigue
    • Lack of appetite
    • Shortness of breath, worsening over time
    • Weight loss
  1. There are over 200 types of pulmonary fibrosis.
    Pulmonary fibrosis is actually an umbrella term for about 200 different types of lung diseases that all appear to function similarly.

  2. The exact cause of pulmonary fibrosis is unknown.
    While the cause is unknown, there are known risk factors. Like many other chronic lung conditions, pulmonary fibrosis can result from long-term exposure to lung irritants like toxic fumes, chemicals, asbestos, silica or coal dust, inorganic dust or tobacco smoke. Consistent heartburn and certain medications are also a known risk factor. When doctors are unsure of the cause, it is deemed idiopathic pulmonary fibrosis.

  3. Approximately 5 million people across the world have pulmonary fibrosis.
    While this is a relatively small number, it means somewhere between 13 and 20 out of every 100,000 people in the world suffer from pulmonary fibrosis, and approximately 30,000 to 50,000 new cases of pulmonary fibrosis are diagnosed each year. Approximately 100,000 people in the Unites States have pulmonary fibrosis.

  4. Pulmonary fibrosis is more prevalent in men than in women.
    In fact, men are nearly twice as likely to be diagnosed with pulmonary fibrosis than women. Women typically have a better prognosis than men.

  5. Pulmonary fibrosis primarily affects people over the age of 50.
    Unfortunately, the older the patient is, the worse their prognosis generally is. As with other degenerative diseases, early diagnosis is key to a better prognosis.

  6. Several tests are required to diagnose pulmonary fibrosis.
    It is necessary to diagnose the type of pulmonary fibrosis the patient has in order to treat it properly. As a result, if a doctor suspects the patient might have pulmonary fibrosis, they will take a full medical history and administer a number of different tests. Those tests could include:
    • Biopsies
    • Blood work
    • Bronchoscopies
    • Imaging tests
    • Lung function tests
    • Physical exams
  1. Pulmonary fibrosis is often seen in conjunction with collagen vascular diseases.
    Collagen vascular diseases like rheumatoid arthritis, scleroderma, Sjögren’s syndrome and systemic lupus are often present in patients diagnosed with pulmonary fibrosis, but the exact reason is not currently known.
  1. About 90% of patients with pulmonary fibrosis also have GERD
    Gastroesophageal reflux disease, or GERD, is a condition in which stomach acid comes back up the esophagus, which causes burning in the throat and heartburn. The reason for the connection is unknown.

  2. Pulmonary fibrosis could be hereditary.

In about 10 to 15% of cases of idiopathic pulmonary fibrosis, there is a family history. This suggests a genetic predisposition, and research has uncovered a number of genes that may be responsible for the development of pulmonary fibrosis. However, additional study is needed to fully understand how each gene is involved. 

  1. On average, pulmonary fibrosis patients lose between 150m and 200ml of lung capacity per year.
    As the disease continues to advance, the scar tissue in the lungs continues to build, decreasing the lungs’ ability to expand and contract, and decreasing the lungs’ overall capacity. The patient experiences worsening symptoms and more breathlessness.

  2. Treatments for pulmonary fibrosis can slow the progression of the disease and ease symptoms.
    Doctors typically recommend a number of different treatment options, including pulmonary rehabilitation, lifestyle changes to adjust to decreased lung capacity, oxygen therapy to improve oxygen intake and prescription medications that may help control the progression of pulmonary fibrosis.
  1. There are two medications that have been shown to slow the progression of pulmonary fibrosis.
    Two medications, Ofev and Esbriet, have been shown to reduce exacerbations and slow the advancement of the disease when given to patients with mild to moderate cases of pulmonary fibrosis.

  2. Some pulmonary fibrosis patients may be eligible for a lung transplant.
    A lung transplant may be the best available choice for extending the lives of patients with pulmonary fibrosis, but it does have risks. The risk of complications is high, and the risk of organ rejection or infection, as a result of medication given to avoid rejection, is high.

    Pulmonary fibrosis is currently the leading cause for lung transplants. As of 2013, nearly half of all donated lungs went to lung transplants for pulmonary fibrosis patients. The most current techniques mean that even patients over 65 may still be able to have successful lung transplants.

  3. There is no cure for pulmonary fibrosis.
    While pulmonary fibrosis can be treated, it cannot be cured. With treatment, symptoms can be minimized to increase the patient’s quality of life. However, even with treatment, the life expectancy after a pulmonary fibrosis diagnosis is three to five years. This is why it is so important to raise awareness for pulmonary fibrosis and encourage the search for new treatments and potential cures.

While the search for cures continues, many treatments can improve patients’ quality of life. One common treatment is oxygen therapy.

How Oxygen Therapy Can Help Pulmonary Fibrosis Patients

The majority of pulmonary fibrosis patients will require oxygen therapy at some point. When and how frequently you need oxygen depends on the severity of your pulmonary fibrosis and your unique symptoms. When it is time to discuss treatment options, your doctor will monitor your oxygen levels and help you decide the right amount of supplemental oxygen for you. Regardless of when you need it, oxygen therapy may allow you to do more than you can without it. Since one of the primary symptoms of pulmonary fibrosis is shortness of breath, oxygen treatments can improve your breathing and your oxygen intake, helping you feel more energized and better able to complete daily activities. Using a portable oxygen concentrator improves that experience even more.

Inogen developed our portable oxygen concentrators to help improve the freedom, independence and mobility of patients using our products. We believe that oxygen therapy should help improve your life—not leave you strapped to a heavy machine or tank indoors—so our portable oxygen concentrators are small and lightweight, allowing you to bring them with you wherever you go. That means whether you are enjoying lunch out with friends or watching a ball game, you can bring your oxygen treatments with you. Talk to your doctor about whether portable oxygen concentrators fit your oxygen therapy needs, and call Inogen today to find out how our portable oxygen concentrators can help you get the oxygen you need anytime, anywhere, to help relieve the symptoms of your pulmonary fibrosis.














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