Pulmonary Fibrosis

What Is Pulmonary Fibrosis?

If you or someone you love has been diagnosed with pulmonary fibrosis, you may be wondering, “What is pulmonary fibrosis?” Pulmonary fibrosis is a disease where there is scarring of the lungs—called fibrosis—which makes it difficult to breathe. This is because the scarring causes the tissues in the lungs to get thick and stiff and makes it hard to absorb oxygen into the bloodstream. Pulmonary fibrosis (PF) may be difficult to diagnose as the symptoms of PF are similar to other lung diseases. There are many different types of PF. If your doctor suspects you might have PF, it is important to see a specialist to confirm your diagnosis. This will help ensure you are treated for the exact disease you have. There is no cure for pulmonary fibrosis. Current treatments are aimed at slowing the course of the disease, relieving symptoms and helping you stay active and healthy.[1]

What Causes Pulmonary Fibrosis?

The majority of the time, doctors are unable to pinpoint the precise cause of pulmonary fibrosis. There are over 200 different types of PF and in most cases, there’s no known cause. Here is a look at some of the different categories of PF.[2]

  • The most common type of PF is IPF, which stands for idiopathic pulmonary fibrosis. This means this type of PF has no known cause.
    • Approximately 50,000 new cases of IPF are diagnosed each year
    • Most IPF patients first start noticing symptoms between the ages of 50 and 70 years old.
    • It is more common in men, but the number of cases of IPF in women is on the rise.
  • Medical conditions
    • Autoimmune diseases like rheumatoid arthritis, scleroderma or Sjogren’s syndrome.
    • Certain viral infections
    • Gastroesophageal reflux disease (GERD) GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.
  • Familial PF is very rare. PF is considered familial when two or more members within the same family have idiopathic pulmonary fibrosis (IPF) or any other form of idiopathic interstitial pneumonia (IIP). There are genes that have been linked to PF but much is still unknown about this field. If someone in your family has had any type of interstitial lung disease, be sure to speak with your doctor and a genetic counselor about your risk.
  • Exposure to hazardous materials.
    • Occupational exposures such as asbestos or silica.
    • Breathing in bird or animal droppings.
    • Radiation treatments
    • Certain types of medications
    • Cigarette smoking increases a person’s risk of developing PF.

How to Treat Pulmonary Fibrosis

Pulmonary fibrosis treatment depends on the severity of the scarring in the lungs. While the damage to the lungs cannot be reversed and the progression of the disease cannot be stopped, treatment can help ease the symptoms of the disease. Treatments are aimed at improving the patient’s breathing and quality of life.[3]

Depending on your particular disease and your symptoms, your doctor may prescribe any of the following:

  • Lung Transplant: Because a lung transplant is a serious surgery with significant risks and potential complications, this treatment is reserved for patients for whom this is the best chance to improve quality of life.
  • Medication: Your doctor may recommend certain medications, including pirfenidone or nintedanib, which could slow the progression of idiopathic pulmonary fibrosis.
  • Oxygen Therapy: Oxygen treatments could help make breathing easier for you, helping to improve your blood oxygen levels and making sleeping, exercise and daily activities easier.
  • Pulmonary Rehabilitation: Pulmonary rehab can help improve your ability to function day to day while giving you the education you need to manage your symptoms as well as possible. It can include physical and/or breathing exercises, nutritional and medical education and counseling or support groups.

Talk to your doctor about which pulmonary fibrosis treatment is right for you and how you can manage your disease and symptoms most successfully for the best quality of life possible.

Risk Factors of Pulmonary Fibrosis

There are over 200 different kinds of pulmonary fibrosis. However, doctors are often unable to determine the cause. When doctors are unable to find what causes pulmonary fibrosis, it is called idiopathic pulmonary fibrosis. However, there are certain factors that could make you more likely to develop pulmonary fibrosis. Those risk factors can include:[2]

  • Age: Pulmonary fibrosis is more common in middle age and older adults
  • Autoimmune diseases: Certain autoimmune diseases can cause pulmonary fibrosis, including rheumatoid arthritis, scleroderma or Sjogren’s syndrome
  • Certain occupations: If you work in construction, farming or mining, or are exposed to the toxins or dusts listed above with any regularity, your risk is higher
  • Genetics: Pulmonary fibrosis can be inherited
  • GERD: Breathing in stomach acid that has backed up into your throat can cause damage to the lungs
  • History of certain cancer treatments: Radiation treatments that occur in the chest area, as well as certain chemo drugs, can increase your risk
  • Sex: Men are more likely than women to develop pulmonary fibrosis
  • Smoking: Smoking increases your risk of developing this disease 

How Inogen Can Help With Your Pulmonary Fibrosis Treatment

Oxygen therapy is a common treatment for pulmonary fibrosis to help improve oxygen levels and ease feelings of breathlessness, allowing you to go about your daily activities without feeling short of breath or overly fatigued.[3] If your doctor determines that oxygen therapy would benefit you and help you manage your pulmonary fibrosis symptoms, ask if a portable oxygen concentrator meets your oxygen needs. While carrying a heavy, awkward compressed oxygen tank around with you may sound daunting, portable oxygen concentrators, like Inogen One are designed for portability, so you can get the oxygen you need at home and away, all day, every day.

Portable oxygen concentrators are compact and lightweight, designed for your on-the-go lifestyle , so you can generate medical grade oxygen as long as you have a charged battery or access to an AC or DC power source. Contact us today to discover the role an Inogen One portable oxygen concentrator can play in your pulmonary fibrosis plan.


  1. www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/
  2. https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction/types-causes-and-risk-factors
  3. https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/how-is-pulmonary-fibrosis-treated

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