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Pulmonary Fibrosis Stages

Pulmonary Fibrosis Stages

If you’re living with a chronic lung disease like pulmonary fibrosis (PF), interstitial lung disease (ILD) or chronic obstructive pulmonary disease (COPD), you’ve likely heard your doctor talk about stages. For people with COPD, the GOLD System and the BODE Index are often used to place COPD into stages, ranking it by disease severity. The GOLD System uses the forced expiratory volume (FEV1) score from a pulmonary function test to determine the stage of COPD. The BODE Index takes into account multiple test results and procedures to determine a COPD stage. However, for people living with pulmonary fibrosis, the pulmonary fibrosis stages are not as well defined. To help you better understand your condition, here’s what you need to know about pulmonary fibrosis stages and what’s being done to help you breathe easier.

What is pulmonary fibrosis?

If you’ve been diagnosed with pulmonary fibrosis or idiopathic pulmonary fibrosis, you’ve probably heard your doctor talk about lung scarring. In pulmonary fibrosis, the lungs become scarred, which causes the intricate pulmonary passageways to thicken and harden. When the lungs thicken and harden, the free passage of oxygen through the walls of the lungs’ tiny air sacs (alveoli) into the bloodstream becomes obstructed. Because of pulmonary fibrosis disease progression, the resulting lack of oxygen in the bloodstream leaves people with pulmonary fibrosis feeling short of breath even after prolonged rest.

Pulmonary fibrosis affects everyone differently and at different rates of disease progression. For some people, pulmonary fibrosis develops rapidly, and for others it progresses slowly over time.

How are the pulmonary fibrosis stages determined?

In general, having a staging system for a particular disease helps doctors and patients work together to develop a treatment plan. Traditionally, pulmonary fibrosis has been in stages with terms such as mild, early, severe and advanced. These simple pulmonary fibrosis stages are based on pulmonary function test results. While this approach allows doctors to monitor pulmonary fibrosis disease progression and helps them advise patients, the current pulmonary fibrosis stages approach doesn’t take into account certain variables.

Some of the baseline features of the disease that could be useful in developing a better system for pulmonary fibrosis stages include level of dyspnea (trouble breathing), forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide, desaturation during the 6-minute walk test, extent of honeycombing appearance of the lungs of CT scans or the presence of pulmonary hypertension or emphysema.

New developments in pulmonary fibrosis staging

Pulmonary Fibrosis Stages

Doctors and researchers have been developing a new staging system for pulmonary fibrosis. The new pulmonary fibrosis stages system currently in development is called the GAP Index. More sophisticated than the traditional pulmonary fibrosis staging system, the GAP Index is comprised of four predictors: age, recent respiratory hospitalization, baseline FVC and 24-week change in FVC. The sum of the individual scores for each factor is then used to obtain a score.

More recently, doctors and researchers have continued to develop the GAP Index, using a multidimensional risk prediction model and staging system. This Gap Index model also consists of 4 baseline variables: gender, age, and two lung physiology variables (FVC and diffusing capacity of the lung for carbon monoxide). They were able to develop a GAP Index calculator to help doctors and patients better understand pulmonary fibrosis stages, where a patient might fall within those stages and the individual risk for each patient.

There are GAP Index calculators available online. Remember to discuss your GAP Index results with your doctor as well as any changes to your symptoms.

What pulmonary fibrosis treatment options are available?

Now that you know more about the pulmonary fibrosis stages and the new developments in better staging systems, you and your doctor can work together to develop the best treatment plan for you. While there’s not a cure for pulmonary fibrosis, there are treatment options available.

Many doctors prescribe medications, inhalers and oxygen therapy to help people with pulmonary fibrosis manage their symptoms. For many people, alternative treatments, such as cellular therapy, have helped patients with pulmonary fibrosis feel better, breathe easier and enjoy an improved quality of life. You can learn more about cellular therapy options for people with pulmonary fibrosis, interstitial lung disease, COPD and other chronic lung diseases by contacting us at (800) 729-3065.


  1. Lung Institute

    4 months ago


    Thank you for your comments and question. We are sorry to hear about your medical conditions. For people who are nonsmokers who are diagnosed with pulmonary fibrosis, a cause is often difficult to determine. We recently did a blog article about that topic and have linked to it.

    We’re happy to answer your questions about cellular therapy for chronic lung diseases. We have a dedicated medical team who have a wealth of knowledge about cellular therapy, treatment options, candidacy, cost and more. So, feel free to give us a call at (855) 313-1149. We look forward to hearing from you soon.


    The Lung Institute

  2. Patty Grimm

    4 months ago

    I was just diagnosed with Interstitial Pulmonary Fibrosis last week. I have had a chronic cough for 13 months and now having SOB. I survived breast cancer 33 years ago and did receive Radiation and Chemo (5fu, Methotrexate and Cytoxin) for a year. I was also exposed to Legionnaires Disease 2 1/2 years ago. I was just wondering what the cause of this disease came from. Is it hereditary? That’s my main concern. I have 2 adult children and 6 grand children. If there is any study that I can go in to help find what causes this disease, I would gladly do it. I was in a study for the breast cancer in 1985 that did help with future treatments. any information would be greatly appreciated.

  3. Lung Institute

    8 months ago


    There is really no way to determine life expectancy with a lung disease because each case is different. Please take a look at these blog articles to provide you with information. For many of our patients, treatment has helped them feel better and breathe more easily.

    We’re happy to answer your questions about cellular therapy for lung disease. Our dedicated medical team has a wealth of knowledge about cellular therapy, treatment options, candidacy and more. So, feel free to contact us at (855) 313-1149 to speak one-on-one with someone from our medical team over our secure phone line. We look forward to hearing from you soon.


    The Lung Institute

  4. Debbie

    9 months ago

    I have mild fibrosis what age is my life :(

  5. Lung Institute

    9 months ago


    Thank you for the comments. If you are overweight, losing any weight, whether you have COPD or not, will help improve your health.

    Having excess abdominal weight may lower one’s lung function, regardless of a persons age, smoking history, or body mass index, according to a study published in the American Journal of Respiratory and Critical Care Medicine. Researchers found that abdominally obese patients (a 35-inch waist for women and a 40-inch waist for men) had poorer lung function than their slim-waisted counterparts. (And these were healthy, COPD-free people.)

    We’re happy to answer your questions about cellular therapy, so feel free to contact us at (855) 313-1149 to speak one-on-one with a patient coordinator. We look forward to hearing from you soon.


    The Lung Institute

  6. Lung Institute

    9 months ago


    Thank you for your message. You might want to contact one of our patient coordinators. They will be happy to answer your questions about cellular therapy for COPD. Our dedicated medical team has a wealth of knowledge about cellular therapy, treatment options, candidacy finances and more. They may have some ideas on fund raising or clinical trials. So, feel free to contact us at (855) 313-1149 to speak one-on-one with someone from our medical team over our secure phone line. We look forward to hearing from you soon.

    Best Regards,

    The Lung Institute

  7. John Cundiff

    10 months ago

    iam not working and can’t get on social security Disabilty because i’am 60 yrs,old with COPs how can I reach out for help for treatments of ant kid please help me if you have any ideas I can’t afford my inhalers thank you and god bless

  8. Tom

    10 months ago

    Hi I just found out I have copd and I was a smoker for 42 years I quite 7 months ago, I feel so much better but I gained a lot of weight, my friend told me that being heavy makes it hard for me to breath and he said if I lost my belly weight I would be able to breath a lot better, is that true?? Thank you Tom

  9. PB

    2 years ago

    Dear Esai,

    First and foremost, we’re sorry to hear about the challenges your sister has been facing with interstitial lung disease. It’s important to discuss any changes in her symptoms with her doctor and to remain on her current treatment plan that she and her doctor have developed. While it’s true that interstitial lung disease is a progressive disease, there are many treatment options available to help people. If you and your sister are concerned that her current treatment plan isn’t working well, it’s best to discuss this with her doctor. Because her doctor knows her and her health situation well, he or she will be able to best guide you. We’re happy to answer any questions you and your sister have regarding cell therapy, so feel free to contact us at (855) 313-1149 to speak with a patient coordinator. We look forward to hearing from you soon.

    Kind Regards,

    The Lung Institute

  10. Esai

    2 years ago

    My sister who got diagnosed for ILD (Interstitial Lung Disease) due to Systemic Sclerosis/mixed connective tissue disease. Her skin was getting darker and mouth narrowing. Doctor said that her lungs function is 32-38% and recommended Steroids based treatment starting this week. Her body is responding to the medicines. But doctor mentioned that the lung disease is irreversible.

    She is 38 years and has 2 kids and they are in Trichy (In Tamilnadu, India).


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