The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
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
The worsening of a disease/condition over time
A formal documentation when someone else is given the responsibility to manage your affairs and make certain decisions on your behalf
Refers to the lungs
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
A type of high blood pressure that affects the blood vessels to the lungs and the right side of the heart15
Therapy that provides relief from symptoms to help patients live more comfortably with their disease13
Administration of oxygen as a medical intervention11
A small plastic tube or prongs that fit in the nostrils to deliver supplementary oxygen11
Listening to and playing music as a therapy which aims to ease the symptoms of those living with IPF
A sleep disorder characterised by breathing that repeatedly stops and starts during sleep12
A specialist who helps someone recover or live with their symptoms more easily
A machine that removes other gases from the air to provide oxygen for oxygen therapy11
An education and exercise program designed to improve the quality of life for people with lung conditions16
A physician specialised in lung problems (also known as a respirologist)
A product that gives protection against a specific infection
A test used to monitor oxygen levels in a patient’s blood, usually with a non-invasive sensor11
A breathing technique to help control breathlessness and reduce anxiety17
A disease that affects only a small percentage of the population
Something that is associated with an increased risk of disease or infection
A technique that helps to reduce stress and anxiety by helping to understand and manage your emotions
Therapies used alongside conventional treatments that help treat symptoms and may improve overall physical and mental wellbeing
A medical study that investigates how safe and effective a new therapy or technique is for treating a certain disease
A disease or condition that occurs at the same time as another disease or condition
A device to deliver compressed air to improve sleep in people with obstructive sleep apnoea6
A disease where a waxy substance (plaque) builds up inside the coronary arteries, which supply oxygen-rich blood to the heart muscle7
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
Refers to the heart, and blood vessels
A type of medication that aims to slow the scarring and stiffening of lungs to slow disease progression2
Tiny air sacs in the lungs where the exchange of oxygen and carbon dioxide to and from the blood takes place
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
Techniques that involve breathing in a certain way to control breathlessness and strengthen your lungs
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
A lung condition where the air sacs within the lungs (alveoli) become damaged5
Extreme weariness resulting from exertion or illness
Of unknown cause
Rapid and uncontrolled breathing
A disease where there is progressive scarring or thickening of the lungs without a known cause4
The surgical removal of cells or tissue samples from the lung for examination by a pathologist10
The lung tissue becomes thickened and stiff
A test that uses a type of X-ray that produces multiple, detailed images of areas inside the body4
A burning sensation in the chest, which can spread to the throat, along with a sour taste in the mouth
Where inflamed tissue is replaced with scar tissue, making it thicken and become stiffer4
A symptom where the ends of the fingers become wider and rounder8
A digestive disease where stomach acid moves up out of the stomach and irritates the lining of the food pipe (oesophagus)9
A physician specializing in the management of diseases of the digestive system
An event characterized by sudden, severe worsening of symptoms or an increase in disease severity1
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
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- 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
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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].
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2.
Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.
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3.
Wynn TA. Integrating mechanisms of pulmonary fibrosis. J Exp Med. 2011;208(7):1339-1350.
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4.
Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3:8.
Also in this section
Symptoms of pulmonary fibrosis
Pulmonary fibrosis can cause many symptoms, such as breathlessness or a cough. Your treatment team can help manage these symptoms
Diagnosing lung conditions with pulmonary fibrosis
Patients with pulmonary fibrosis may have symptoms that are common in many diseases, meaning diagnosis can take a long time
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
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Recently diagnosed
The time following diagnosis can be difficult, although there are lots of ways to help you deal with your diagnosis
Patient stories
Managing your pulmonary fibrosis
There are steps that can be taken to help manage some of your symptoms of pulmonary fibrosis to help you live life to the fullest