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Treatments

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IPF can be managed using many different treatment strategies, as well as by making some lifestyle changes

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 programme 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 characterised 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 specialising in the management of diseases of the digestive system

View in glossary

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

View in glossary

While there is no cure for IPF, various therapies may help to alleviate your symptoms and slow disease progression.1 Treatment strategies can include medications, and other options such as pulmonary rehabilitation, oxygen therapy and lung transplantation.2


The main goal of your treatment will usually be to control your symptoms and slow disease progression. Your treatment team will be able to work with you to develop a treatment plan that suits your needs. Using both medicinal and non-medicinal treatments can help you to manage the progression of your disease.

Treatment strategies and various medications can help to slow down the disease

  1. Diagram showing lung function
    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 programme 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 characterised 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 specialising in the management of diseases of the digestive system

    View in glossary

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

    View in glossary

    Without treatment patients lose up to 280mL of lung function each year3,4

  2. Diagram showing lung function
    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 programme 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 characterised 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 specialising in the management of diseases of the digestive system

    View in glossary

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

    View in glossary

    The goal of treatment is to slow the loss of lung function3,4

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 programme 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 characterised 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 specialising in the management of diseases of the digestive system

View in glossary

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

View in glossary

It is important to remember that treatment decisions for IPF are personal for each patient. The treatment decision may be affected by factors such as:2

  • Other diseases or disorders the patient may have
  • Other medications you may be taking
  • Treatment side effects
  • The risk of acute exacerbations (a sudden and rapid worsening of your condition)
  • Your age and physical condition
  • Your personal preference about treatment options

It's important that you talk to your treatment team about which therapy options may be right for you.


Currently there are several planned and ongoing clinical trials to test new and promising medications for the treatment of IPF. If you want to know more about clinical trials in IPF, visit the Clinical Trials Register website and ask your treatment team about any trials in your area.

Doctors and nurses discussing x-ray results
Clinical trials
Clinical trials help develop new treatments to find better options for people living with IPF

Key Takeaways

  • There is currently no cure for IPF

  • Treatments are available that can slow disease progression

  • Your treatment team can develop a treatment plan that includes medical and non-medical therapies to manage your condition

Show references Hide references
  1. 1.

    Lipsi R, et al. Severe idiopathic pulmonary fibrosis: A clinical approach. Eur J Intern Med. 2018;50: 20–27.

  2. 2.

    Raghu G, et al. An official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2011; 183: 788–824.

  3. 3.

    King TE, et al. A Phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. New Engl J Med 2014;370:2083-2092.

  4. 4.

    Richeldi L, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. New Engl J Med 2014;370:2071–2082.

Also in this section

It is important to stay positive, to find the doctor that you trust in, to have a good support from the family, stay active, have a healthy food intake, and so overall it is important to create a positive atmosphere

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