Female doctor explaining information to male patient

What is IPF?

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IPF is a rare, progressive disease that causes a build-up of scarring in the lungs

An operation to replace a patient’s diseased lungs with lungs from a donor
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The worsening of a disease/condition over time

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

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Refers to the lungs

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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

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

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

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Administration of oxygen as a medical intervention11

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

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

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

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

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

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

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

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A product that gives protection against a specific infection

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

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A breathing technique to help control breathlessness and reduce anxiety17

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A disease that affects only a small percentage of the population

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

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

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

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

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

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

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

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

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Refers to the heart, and blood vessels

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

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

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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

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

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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

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

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Extreme weariness resulting from exertion or illness

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Of unknown cause

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Rapid and uncontrolled breathing

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

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

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The lung tissue becomes thickened and stiff

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

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

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

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

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

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

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

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IPF stands for Idiopathic Pulmonary Fibrosis, a rare condition that affects only 10 – 60 people in 100,000 each year.1

  

Fibrosis is the medical term for a build-up of scar tissue. In someone with IPF, pulmonary (or lung) tissue, or the lungs become scarred, thickened and stiff. IPF is a progressive condition, which means it gets worse over time.2

 

Tiny air sacs (known as alveoli) and blood vessels of the lungs are responsible for providing the oxygen to all parts of the body, but as scarring of the lung tissue and air sacs develops, it becomes harder for oxygen to pass through the lungs and into the bloodstream. This results in a shortness of breath and eventually leads to the body's organs not getting enough oxygen to work properly.1,3
 

Environmental pollutants and certain medicines can cause IPF. However, in most cases the exact cause of this lung scarring is never established. This means the pulmonary fibrosis is idiopathic.4 Certain factors, such as cigarette smoking, some viral infections, genetics and GERD (gastro-oesophageal reflux disease) may increase the risk of IPF.5

Key Takeaways

    • IPF is a rare condition, with around 10 – 60 people per 100,000 diagnosed each year
  • In IPF, the lungs become scarred making it harder for oxygen to enter the blood

  • ​There is no exact cause of IPF known

Show references Hide references
  1. 1.
    1. Martinez FJ, et al. Idiopathic pulmonary fibrosis. Nature Reviews Disease Primers; 2017; 3:17074.
  2. 2.

    Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824.

  3. 3.

    Woodcock HV, et al. The treatment of idiopathic pulmonary fibrosis. F1000prime Rep 2014; 6:16.

  4. 4.

    Meltzer EB, et al. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008; 3:8.

  5. 5.

    Fernandez IE, et al. New cellular and molecular mechanisms of lung injury and fibrosis idiopathic pulmonary fibrosis. Lancet 2012; 380:680–688.

Also in this section

The typical and most frequent signs and symptoms of IPF are shortness of breath and cough. And shortness of breath starts with exercise and then will increase over time

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