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Understanding pulmonary fibrosis

Disease progression

Many types of pulmonary fibrosis (PF) are progressive conditions, such as idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF). This means the lung tissue will become thicker and more scarred over time. It’s important to understand that progression is about the disease itself, not just the symptoms you may feel.2-5

PF iceberg

Think of PF as an iceberg


On the surface, there are symptoms that may be hard to ignore. But below the surface, IPF and PPF cause something even more serious— inflammation and scarring of the lungs that get worse over time.2,3,6-8

As IPF and PPF progress, symptoms get worse and lungs don’t function as well as they should—and this damage cannot be undone.3
 

What are the signs of disease progression?

Lung image representing lung function decline
Lung function continues to decline2,4

Your doctor can perform tests to measure this, such as forced vital capacity (FVC)9,10

Warning for worsening PF symptoms
Worsening symptoms2,4

This may include shortness of breath, cough, or fatigue3

Lung with scarring
Scarring of the lungs gets worse2,4

Your doctor can see scarring with a high-resolution CT scan10

How does disease progression affect me?


The progression of PF will cause your lung function to decline. Looking at this graph, you can see that, in a person with healthy lungs, lung function gradually declines as the person gets older.3

In a person who has IPF or PPF, disease progression causes lung function decline to happen more quickly, which makes breathing more difficult. Additionally, some people living with IPF or PPF may experience an acute exacerbation—a sudden decline in lung function along with worsening lung scarring.3,5,7,11

Graph comparing normal vs PF lung function decline

As PF gets worse, it can also:

Warning for worsening PF symptoms
Make your symptoms worse2,13
Compressed gas
Cause you to need extra oxygen (supplemental oxygen)10
Star
Affect your quality of life7
Brain
Impact your mental health14
Down Arrow
Shorten your life expectancy5

Progression will make breathing challenging and, as a result, you may feel more tired and have trouble moving around. This can make it harder to reach daily and long-term goals, like working, exercising, traveling, or spending time with your loved ones.3,5,7

Important

Progression is the hidden force behind your disease, so it is important to treat progression urgently to protect as much lung function as possible. A treatment that slows progression could help you5,10:

- Keep your symptoms from worsening
- Hold onto lung function longer
- Enjoy more moments with loved ones

I have sarcoidosis

Your voice matters when choosing your treatment plan

Talking to your care team about your treatment goals can help inform critical decisions about the best options. It’s important to know how well certain options may work and how they may affect your body. 

In conversations with your care team, you should also ask questions, raise concerns, and speak honestly about how treatment will fit into all aspects of your life—physically, mentally, or financially.

Setting goals and following a care plan can help you feel more in control of PF.

Talk to your doctor about treatment options. A good treatment plan may help you slow the progression of PF. There are many different treatment options.10

The progression of PF can come with difficult emotions.

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Abbreviations: CT, computed tomography; FVC, forced vital capacity; IPF, idiopathic pulmonary fibrosis; PF, pulmonary fibrosis; PPF, progressive pulmonary fibrosis.

  1. Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3:8.

  2. Cottin V. Criteria for progressive pulmonary fibrosis: getting the horse ready for the cart. Am J Respir Crit Care Med. 2023;207(1):11-13.

  3. Mayo Clinic Staff. Pulmonary fibrosis. Updated February 15, 2024. Accessed September 2, 2025. https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis

  4. American Lung Association. Pulmonary fibrosis progression and exacerbation. Updated June 7, 2024. Accessed September 2, 2025. https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/living-well/progression-and-exacerbation

  5. Cleveland Clinic. Pulmonary fibrosis. Last reviewed April 5, 2021. Accessed September 2, 2025. https://my.clevelandclinic.org/health/diseases/21431-pulmonary-fibrosis

  6. Rajan SK, Cottin V, Dhar R, et al. Progressive pulmonary fibrosis: an expert group consensus statement. Eur Respir J. 2023;61(3):2103187.

  7. Asthma + Lung UK. What is pulmonary fibrosis? Updated June 1, 2022. Accessed September 2, 2025. https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis/what-pulmonary-fibrosis

  8. National Heart, Lung, and Blood Institute. What is idiopathic pulmonary fibrosis? National Institutes of Health. Updated June 26, 2023. Accessed September 2, 2025. https://www.nhlbi.nih.gov/health/idiopathic-pulmonary-fibrosis

  9. Cleveland Clinic. Lung diffusion testing (DLCO). Last reviewed February 8, 2024. Accessed September 2, 2025. https://my.clevelandclinic.org/health/diagnostics/15445-lung-diffusion-testing-dlco 

  10. Mayo Clinic Staff. Pulmonary fibrosis: diagnosis and treatment. Updated February 15, 2024. Accessed September 2, 2025. https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/diagnosis-treatment

  11. Wong AW, Ryerson CJ, Guler SA. Progression of fibrosing interstitial lung disease. Respir Res. 2020;21(1):32.

  12. Podolanczuk AJ, Thomson CC, Remy-Jardin M, et al. Idiopathic pulmonary fibrosis: state of the art for 2023. Eur Respir J. 2023;61(4):2200957.

  13. Lung Foundation Australia. Managing progression in pulmonary fibrosis. Published February 2024. Accessed September 2, 2025. https://lungfoundation.com.au

  14. American Lung Association. Coping with stress and emotions from pulmonary fibrosis. Updated June 7, 2024. Accessed September 2, 2025. https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/living-well/coping-with-stress