What is pulmonary fibrosis?
Pulmonary fibrosis is the medical term for when healthy lung tissue is replaced with scar tissue, making the lungs thicken and become stiff
Pulmonary fibrosis is the medical term for a build-up of scar tissue in the lung. In someone with pulmonary fibrosis, the lungs become scarred, thickened and stiff.1,2
Pulmonary fibrosis occurs in many types of interstitial lung diseases (ILDs). ‘Interstitial’ means the disease affects the interstitium, the lace-like network of tissue that supports the air sacs in your lungs. There are more than 200 different ILDs.1 Find out more about the different conditions associated with pulmonary fibrosis.
Tiny air sacs (known as alveoli) and blood vessels of the lungs are responsible for providing oxygen to all parts of the body. As pulmonary fibrosis (scarring of the lung tissue and air sacs) develops, it becomes harder for oxygen to pass through the lungs and into the bloodstream. The symptoms of pulmonary fibrosis vary from person to person, but in some patients, worsening of the disease can result in shortness of breath and eventually lead to the body's organs not getting enough oxygen to work properly.2,3 It's important to be aware of all the common symptoms of pulmonary fibrosis.
Resources for you
Pulmonary fibrosis pocket doktor
Questions to ask your doctor or nurse
Pulmonary fibrosis eBook
Although many ILDs are characterised by pulmonary fibrosis, some ILDs are characterised by inflammation.1
In some types of interstitial lung diseases, the exact cause of this lung scarring can be identified but for many types, a definite cause is never established.1,4
Key takeaways
ILD is a term that covers many different conditions that are caused by scarring of the lung tissue
Pulmonary fibrosis is a term that means scarring of the lungs
In some patients, the scarring of the lung tissue may worsen over time
British Lung Foundation. What is pulmonary fibrosis? Available at:
https://www.blf.org.uk/support-for-you/pulmonary-fibrosis/what-is-pulmonary-fibrosis
[Accessed March 2022].
Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.
Wynn TA. Integrating mechanisms of pulmonary fibrosis. J Exp Med. 2011;208(7):1339-1350.
Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3:8.
Also in this section
Symptoms of pulmonary fibrosis
Diagnosing lung conditions with pulmonary fibrosis
“The typical and most frequent signs and symptoms of pulmonary fibrosis are shortness of breath and cough. And shortness of breath starts with exercise and then will increase over time”