What is pulmonary fibrosis?

2 min read |

Pulmonary fibrosis is the medical term for when healthy lung tissue is replaced with scar tissue making the lungs thicken and become stiff

An operation to replace a patient’s diseased lungs with lungs from a donor
View in glossary

The worsening of a disease/condition over time

View in glossary

A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf

View in glossary

Refers to the lungs

View in glossary

A group of tests used to check how well the lungs take in and release air and how well they supply oxygen to the rest of the body14

View in glossary

A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15

View in glossary

Therapy that provides relief from symptoms to help patients live more comfortably with their disease13

View in glossary

Administration of oxygen as a medical intervention11

View in glossary

A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11

View in glossary

Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF

View in glossary

A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12

View in glossary

A specialist who helps someone recover or live with their symptoms more easily

View in glossary

A machine that removes other gases from the air to provide oxygen for oxygen therapy11

View in glossary

An education and exercise program designed to improve the quality of life for people with lung conditions16

View in glossary

A physician specialised in lung problems (also known as a respirologist)

View in glossary

A product that gives protection against a specific infection

View in glossary

A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11

View in glossary

A breathing technique to help control breathlessness and reduce anxiety17

View in glossary

A disease that affects only a small percentage of the population

View in glossary

Something that is associated with an increased risk of disease or infection

View in glossary

A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions

View in glossary

Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing

View in glossary

A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease

View in glossary

A disease or condition that occurs at the same time as another disease or condition

View in glossary

A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6

View in glossary

A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7

View in glossary

A type of chronic disease that typically worsens over time and is characterized by long-term breathing problems and poor airflow. Chronic bronchitis and emphysema are older terms used for COPD5

View in glossary

Refers to the heart, and blood vessels

View in glossary

A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2

View in glossary

Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place

View in glossary

A test that shows how well the lungs are working by measuring how much oxygen and carbon dioxide is in the blood. This test requires that a small volume of blood be drawn from the patient3

View in glossary

Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs

View in glossary

A procedure in which a bronchoscope (a medical instrument like a tube) is passed through the mouth or nose into the lung and fluid is squirted into a small part of the lung and then collected for examination4

View in glossary

A lung condition where the air sacs within the lungs (alveoli) become damaged5

View in glossary

Extreme weariness resulting from exertion or illness

View in glossary

Of unknown cause

View in glossary

Rapid and uncontrolled breathing

View in glossary

A disease where there is progressive scarring or thickening of the lungs without a known cause4

View in glossary

The surgical removal of cells or tissue samples from the lung for examination by a pathologist10

View in glossary

The lung tissue becomes thickened and stiff

View in glossary

A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4

View in glossary

A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth

View in glossary

Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4

View in glossary

A symptom where the ends of the fingers become wider and rounder8

View in glossary

A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9

View in glossary

A physician specializing in the management of diseases of the digestive system

View in glossary

An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1

View in glossary

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

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. In some patients, pulmonary fibrosis keeps worsening, this results in shortness of breath and eventually leads to the body's organs not getting enough oxygen to work properly.2,3

Although many ILDs are characterized by pulmonary fibrosis, some ILDs are characterized 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

Show references Hide references
  1. 1.
    1. British Lung Foundation. What is pulmonary fibrosis? Available at: https://www.blf.org.uk/support-for-you/pulmonary-fibrosis/what-is-pulmon.... [Accessed April 2019].
  2. 2.
    1. Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.
  3. 3.
    1. Wynn TA. Integrating mechanisms of pulmonary fibrosis. J Exp Med. 2011;208(7):1339-1350.
  4. 4.
    1. Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3:8.
Also in this section
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
You may also be interested in

This site uses cookies to improve your browsing experience. By using this site, you agree to their use. Cookie Information