Older male patient chatting with nurse

Progression of IPF

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

Happens when stomach acid travels up into and irritates the esophagus (tube connecting the throat with the stomach)

View in glossary

The worsening of a disease/condition over time

View in glossary

A breathing technique to help control breathlessness and reduce anxiety

View in glossary

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

View in glossary

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

View in glossary

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

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 body

View in glossary

Scarring in the lungs 

View in glossary

A lung problem when the pressure in the blood vessels in the lungs gets too high 

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

A sudden decrease in blood supply to the extremities, which causes fingers and/or toes to get numb, cold and painful, with changes to skin colour (usually white to blue/purple then to red) 

View in glossary

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

View in glossary

Administration of oxygen as a medical intervention

View in glossary

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

View in glossary

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

View in glossary

A sleep disorder characterized by breathing that repeatedly stops and starts during sleep 

View in glossary

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

View in glossary

A painless test that uses a microscope or a small camera to check the tiny blood vessels at the base of the nail 

View in glossary

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

View in glossary

A disease that affects only a small percentage of the population

View in glossary

Relating to the act of breathing 

View in glossary

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

View in glossary

Patients who have systemic sclerosis that also exhibit features of another autoimmune connective tissue disease at the same time such as rheumatoid arthritis 

View in glossary

A product that gives protection against a specific infection

View in glossary

Small red spots caused by widened blood vessels usually on the hands and face 

View in glossary

Something that has an effect on the whole body and not just one part of it 

View in glossary

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

View in glossary

A set of medical signs and symptoms that consistently occur together, or a particular disease or disorder that is characterized by a set of associated symptoms 

View in glossary

Any feeling of illness or physical or mental change that is caused by a particular disease 

View in glossary

The measurement of breathing capacity using a device called a spirometer 

View in glossary

The hardening or thickening of tissues in the body 

View in glossary

The sclerosis (hardening) of the skin (derma), specifically. However, scleroderma is the term that is often used when discussing the disorder known as systemic sclerosis. Systemic sclerosis is a rare, chronic, autoimmune rheumatic disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities 

View in glossary

Thickening and tightening of the skin on the fingers or toes 

View in glossary

A long-term autoimmune disease that causes pain, swelling and deformity of the joints 

View in glossary

Magnetic Resonance Imaging. A scan that uses a magnet and radio waves to look at organs and structures inside your body

View in glossary

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

View in glossary

Refers to the heart, and blood vessels.

View in glossary

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

View in glossary

Tissue that supports and connects other tissues and parts of the body, e.g., tendons, cartilage, bone 

View in glossary

A component of connective tissue 

View in glossary

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

View in glossary

A type of chronic disease that typically worsens over time and is characterized by long-term breathing problems and poor airflow.

View in glossary

Hard lumps (made from calcium) that develop in the innermost layer of skin or in muscle 

View in glossary

Microscope or a small camera used to look at your skin 

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 examination

View in glossary

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

View in glossary

Disease where the immune system acts against the person’s own body. ‘Auto’ = self; ‘Immune’ = protection against

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 patient

View in glossary

Little proteins that form a key part of your body’s defense against foreign substances like bacteria and viruses. They search for and attach themselves to these invaders, helping your immune system find and get rid of them 

View in glossary

A type of medication that prevents or reduces scarring.

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

Computed Tomography (CT). A type of x-ray that provides more detailed imaging than normal x-rays are capable of providing

View in glossary

Test which uses soundwaves to look at the structure of your heart and how well it can pump your blood

View in glossary

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

View in glossary

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

View in glossary

The lace-like network of tissue that supports the air sacs. 

View in glossary

A group of lung disorders that are characterized by scarring of the deep lung tissue that can cause breathing issues such as shortness of breath and loss of functioning alveoli 

View in glossary

A normal response from your immune system, causing the affected part of the body to become red, swollen, hot, and often painful, in reaction to injury or infection 

View in glossary

Your body’s defense system against foreign organisms or substances 

View in glossary

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

View in glossary

Of unknown cause

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

Test which records electrical signals from your heart, to see how well your heart is beating 

View in glossary

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

View in glossary

The total amount of air exhaled during forced expiratory volume (FEV) test 

View in glossary

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

View in glossary

A type of cell found in connective tissue that produces collagen 

View in glossary

Extreme weariness resulting from exertion or illness

View in glossary

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

View in glossary

Measuring the progression of IPF

 

Your doctor, supported by a wider treatment team of experienced specialists, will use your medical history, a physical examination and a combination of different tests to find out how advanced your disease is. Your doctor will then tailor the best treatment plan for you.1,2

 

Unlike many other chronic diseases, there are no clear-cut "stages" of IPF. The course of IPF is more commonly referred to as “early” or “advanced”. Everyone experiences it differently; some people can live for several years after diagnosis.2

 

Please visit the taking care of yourself page for more information about some of the treatments and health advice you can discuss with your doctor.

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

Happens when stomach acid travels up into and irritates the esophagus (tube connecting the throat with the stomach)

View in glossary

The worsening of a disease/condition over time

View in glossary

A breathing technique to help control breathlessness and reduce anxiety

View in glossary

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

View in glossary

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

View in glossary

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

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 body

View in glossary

Scarring in the lungs 

View in glossary

A lung problem when the pressure in the blood vessels in the lungs gets too high 

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

A sudden decrease in blood supply to the extremities, which causes fingers and/or toes to get numb, cold and painful, with changes to skin colour (usually white to blue/purple then to red) 

View in glossary

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

View in glossary

Administration of oxygen as a medical intervention

View in glossary

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

View in glossary

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

View in glossary

A sleep disorder characterized by breathing that repeatedly stops and starts during sleep 

View in glossary

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

View in glossary

A painless test that uses a microscope or a small camera to check the tiny blood vessels at the base of the nail 

View in glossary

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

View in glossary

A disease that affects only a small percentage of the population

View in glossary

Relating to the act of breathing 

View in glossary

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

View in glossary

Patients who have systemic sclerosis that also exhibit features of another autoimmune connective tissue disease at the same time such as rheumatoid arthritis 

View in glossary

A product that gives protection against a specific infection

View in glossary

Small red spots caused by widened blood vessels usually on the hands and face 

View in glossary

Something that has an effect on the whole body and not just one part of it 

View in glossary

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

View in glossary

A set of medical signs and symptoms that consistently occur together, or a particular disease or disorder that is characterized by a set of associated symptoms 

View in glossary

Any feeling of illness or physical or mental change that is caused by a particular disease 

View in glossary

The measurement of breathing capacity using a device called a spirometer 

View in glossary

The hardening or thickening of tissues in the body 

View in glossary

The sclerosis (hardening) of the skin (derma), specifically. However, scleroderma is the term that is often used when discussing the disorder known as systemic sclerosis. Systemic sclerosis is a rare, chronic, autoimmune rheumatic disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities 

View in glossary

Thickening and tightening of the skin on the fingers or toes 

View in glossary

A long-term autoimmune disease that causes pain, swelling and deformity of the joints 

View in glossary

Magnetic Resonance Imaging. A scan that uses a magnet and radio waves to look at organs and structures inside your body

View in glossary

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

View in glossary

Refers to the heart, and blood vessels.

View in glossary

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

View in glossary

Tissue that supports and connects other tissues and parts of the body, e.g., tendons, cartilage, bone 

View in glossary

A component of connective tissue 

View in glossary

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

View in glossary

A type of chronic disease that typically worsens over time and is characterized by long-term breathing problems and poor airflow.

View in glossary

Hard lumps (made from calcium) that develop in the innermost layer of skin or in muscle 

View in glossary

Microscope or a small camera used to look at your skin 

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 examination

View in glossary

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

View in glossary

Disease where the immune system acts against the person’s own body. ‘Auto’ = self; ‘Immune’ = protection against

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 patient

View in glossary

Little proteins that form a key part of your body’s defense against foreign substances like bacteria and viruses. They search for and attach themselves to these invaders, helping your immune system find and get rid of them 

View in glossary

A type of medication that prevents or reduces scarring.

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

Computed Tomography (CT). A type of x-ray that provides more detailed imaging than normal x-rays are capable of providing

View in glossary

Test which uses soundwaves to look at the structure of your heart and how well it can pump your blood

View in glossary

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

View in glossary

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

View in glossary

The lace-like network of tissue that supports the air sacs. 

View in glossary

A group of lung disorders that are characterized by scarring of the deep lung tissue that can cause breathing issues such as shortness of breath and loss of functioning alveoli 

View in glossary

A normal response from your immune system, causing the affected part of the body to become red, swollen, hot, and often painful, in reaction to injury or infection 

View in glossary

Your body’s defense system against foreign organisms or substances 

View in glossary

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

View in glossary

Of unknown cause

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

Test which records electrical signals from your heart, to see how well your heart is beating 

View in glossary

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

View in glossary

The total amount of air exhaled during forced expiratory volume (FEV) test 

View in glossary

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

View in glossary

A type of cell found in connective tissue that produces collagen 

View in glossary

Extreme weariness resulting from exertion or illness

View in glossary

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

View in glossary
Survival time

Survival time for IPF can vary from patient-to-patient. IPF may progress quite quickly in some patients, while in others it may change slowly. Some research suggests that half of people with IPF live for about 3 to 5 years after diagnosis. However, these estimates were based on information provided before new treatments for IPF were available.1,3

 

With new antifibrotic treatments for IPF, survival time may be increased. Although the new treatments cannot reverse the scarring on the lungs, they may slow the progression of the disease.4-6

 

In addition to treatments, your survival time can be influenced by your age, degree of breathlessness and how the disease affects your body individually.3

 

 

Couple walking by lake

Your wellbeing

Carers have a demanding role, but support and resources are available to help you

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

Happens when stomach acid travels up into and irritates the esophagus (tube connecting the throat with the stomach)

View in glossary

The worsening of a disease/condition over time

View in glossary

A breathing technique to help control breathlessness and reduce anxiety

View in glossary

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

View in glossary

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

View in glossary

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

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 body

View in glossary

Scarring in the lungs 

View in glossary

A lung problem when the pressure in the blood vessels in the lungs gets too high 

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

A sudden decrease in blood supply to the extremities, which causes fingers and/or toes to get numb, cold and painful, with changes to skin colour (usually white to blue/purple then to red) 

View in glossary

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

View in glossary

Administration of oxygen as a medical intervention

View in glossary

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

View in glossary

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

View in glossary

A sleep disorder characterized by breathing that repeatedly stops and starts during sleep 

View in glossary

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

View in glossary

A painless test that uses a microscope or a small camera to check the tiny blood vessels at the base of the nail 

View in glossary

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

View in glossary

A disease that affects only a small percentage of the population

View in glossary

Relating to the act of breathing 

View in glossary

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

View in glossary

Patients who have systemic sclerosis that also exhibit features of another autoimmune connective tissue disease at the same time such as rheumatoid arthritis 

View in glossary

A product that gives protection against a specific infection

View in glossary

Small red spots caused by widened blood vessels usually on the hands and face 

View in glossary

Something that has an effect on the whole body and not just one part of it 

View in glossary

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

View in glossary

A set of medical signs and symptoms that consistently occur together, or a particular disease or disorder that is characterized by a set of associated symptoms 

View in glossary

Any feeling of illness or physical or mental change that is caused by a particular disease 

View in glossary

The measurement of breathing capacity using a device called a spirometer 

View in glossary

The hardening or thickening of tissues in the body 

View in glossary

The sclerosis (hardening) of the skin (derma), specifically. However, scleroderma is the term that is often used when discussing the disorder known as systemic sclerosis. Systemic sclerosis is a rare, chronic, autoimmune rheumatic disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities 

View in glossary

Thickening and tightening of the skin on the fingers or toes 

View in glossary

A long-term autoimmune disease that causes pain, swelling and deformity of the joints 

View in glossary

Magnetic Resonance Imaging. A scan that uses a magnet and radio waves to look at organs and structures inside your body

View in glossary

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

View in glossary

Refers to the heart, and blood vessels.

View in glossary

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

View in glossary

Tissue that supports and connects other tissues and parts of the body, e.g., tendons, cartilage, bone 

View in glossary

A component of connective tissue 

View in glossary

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

View in glossary

A type of chronic disease that typically worsens over time and is characterized by long-term breathing problems and poor airflow.

View in glossary

Hard lumps (made from calcium) that develop in the innermost layer of skin or in muscle 

View in glossary

Microscope or a small camera used to look at your skin 

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 examination

View in glossary

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

View in glossary

Disease where the immune system acts against the person’s own body. ‘Auto’ = self; ‘Immune’ = protection against

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 patient

View in glossary

Little proteins that form a key part of your body’s defense against foreign substances like bacteria and viruses. They search for and attach themselves to these invaders, helping your immune system find and get rid of them 

View in glossary

A type of medication that prevents or reduces scarring.

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

Computed Tomography (CT). A type of x-ray that provides more detailed imaging than normal x-rays are capable of providing

View in glossary

Test which uses soundwaves to look at the structure of your heart and how well it can pump your blood

View in glossary

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

View in glossary

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

View in glossary

The lace-like network of tissue that supports the air sacs. 

View in glossary

A group of lung disorders that are characterized by scarring of the deep lung tissue that can cause breathing issues such as shortness of breath and loss of functioning alveoli 

View in glossary

A normal response from your immune system, causing the affected part of the body to become red, swollen, hot, and often painful, in reaction to injury or infection 

View in glossary

Your body’s defense system against foreign organisms or substances 

View in glossary

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

View in glossary

Of unknown cause

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

Test which records electrical signals from your heart, to see how well your heart is beating 

View in glossary

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

View in glossary

The total amount of air exhaled during forced expiratory volume (FEV) test 

View in glossary

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

View in glossary

A type of cell found in connective tissue that produces collagen 

View in glossary

Extreme weariness resulting from exertion or illness

View in glossary

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

View in glossary

There are practical issues to think about when you know you have a life-limiting condition. These might include drawing up a will, managing your finances and deciding on the type of supportive care you would like to receive as the disease progresses.

 

Please visit the planning for the future page to learn more about some of the practical issues you may need assistance with.

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

Happens when stomach acid travels up into and irritates the esophagus (tube connecting the throat with the stomach)

View in glossary

The worsening of a disease/condition over time

View in glossary

A breathing technique to help control breathlessness and reduce anxiety

View in glossary

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

View in glossary

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

View in glossary

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

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 body

View in glossary

Scarring in the lungs 

View in glossary

A lung problem when the pressure in the blood vessels in the lungs gets too high 

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

A sudden decrease in blood supply to the extremities, which causes fingers and/or toes to get numb, cold and painful, with changes to skin colour (usually white to blue/purple then to red) 

View in glossary

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

View in glossary

Administration of oxygen as a medical intervention

View in glossary

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

View in glossary

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

View in glossary

A sleep disorder characterized by breathing that repeatedly stops and starts during sleep 

View in glossary

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

View in glossary

A painless test that uses a microscope or a small camera to check the tiny blood vessels at the base of the nail 

View in glossary

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

View in glossary

A disease that affects only a small percentage of the population

View in glossary

Relating to the act of breathing 

View in glossary

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

View in glossary

Patients who have systemic sclerosis that also exhibit features of another autoimmune connective tissue disease at the same time such as rheumatoid arthritis 

View in glossary

A product that gives protection against a specific infection

View in glossary

Small red spots caused by widened blood vessels usually on the hands and face 

View in glossary

Something that has an effect on the whole body and not just one part of it 

View in glossary

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

View in glossary

A set of medical signs and symptoms that consistently occur together, or a particular disease or disorder that is characterized by a set of associated symptoms 

View in glossary

Any feeling of illness or physical or mental change that is caused by a particular disease 

View in glossary

The measurement of breathing capacity using a device called a spirometer 

View in glossary

The hardening or thickening of tissues in the body 

View in glossary

The sclerosis (hardening) of the skin (derma), specifically. However, scleroderma is the term that is often used when discussing the disorder known as systemic sclerosis. Systemic sclerosis is a rare, chronic, autoimmune rheumatic disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities 

View in glossary

Thickening and tightening of the skin on the fingers or toes 

View in glossary

A long-term autoimmune disease that causes pain, swelling and deformity of the joints 

View in glossary

Magnetic Resonance Imaging. A scan that uses a magnet and radio waves to look at organs and structures inside your body

View in glossary

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

View in glossary

Refers to the heart, and blood vessels.

View in glossary

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

View in glossary

Tissue that supports and connects other tissues and parts of the body, e.g., tendons, cartilage, bone 

View in glossary

A component of connective tissue 

View in glossary

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

View in glossary

A type of chronic disease that typically worsens over time and is characterized by long-term breathing problems and poor airflow.

View in glossary

Hard lumps (made from calcium) that develop in the innermost layer of skin or in muscle 

View in glossary

Microscope or a small camera used to look at your skin 

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 examination

View in glossary

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

View in glossary

Disease where the immune system acts against the person’s own body. ‘Auto’ = self; ‘Immune’ = protection against

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 patient

View in glossary

Little proteins that form a key part of your body’s defense against foreign substances like bacteria and viruses. They search for and attach themselves to these invaders, helping your immune system find and get rid of them 

View in glossary

A type of medication that prevents or reduces scarring.

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

Computed Tomography (CT). A type of x-ray that provides more detailed imaging than normal x-rays are capable of providing

View in glossary

Test which uses soundwaves to look at the structure of your heart and how well it can pump your blood

View in glossary

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

View in glossary

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

View in glossary

The lace-like network of tissue that supports the air sacs. 

View in glossary

A group of lung disorders that are characterized by scarring of the deep lung tissue that can cause breathing issues such as shortness of breath and loss of functioning alveoli 

View in glossary

A normal response from your immune system, causing the affected part of the body to become red, swollen, hot, and often painful, in reaction to injury or infection 

View in glossary

Your body’s defense system against foreign organisms or substances 

View in glossary

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

View in glossary

Of unknown cause

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

Test which records electrical signals from your heart, to see how well your heart is beating 

View in glossary

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

View in glossary

The total amount of air exhaled during forced expiratory volume (FEV) test 

View in glossary

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

View in glossary

A type of cell found in connective tissue that produces collagen 

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Extreme weariness resulting from exertion or illness

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

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Make sure you speak to your doctor and those close to you about managing practical issues as early as possible. Having plans in place can give you peace of mind and help those assisting with your care.

Key Takeaways

    • Your healthcare team will carry out tests to monitor your disease
    • Survival time from diagnosis can vary from patient to patient, and may be improved with treatments
    • Planning for the future is an important part of living with a life limiting condition
Show references Hide references
  1. 1.
    1. Meltzer EB, et al. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008; 3:8
  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.

    Martinez FJ, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers 2017; 3:17074.

  4. 4.
    1. Fisher M, et al. Predicting life expectancy for pirfenidone in idiopathic pulmonary fibrosis. J Manag Care Spec Pharm 2017; 23: S17-S24.
  5. 5.
    1. Woodcock HV, et al. The treatment of idiopathic pulmonary fibrosis. F1000Prime Rep 2014; 6:16.
  6. 6.
    1. Fleetwood K, et al. Systematic review and network meta-analysis of idiopathic pulmonary fibrosis treatments. J Manag Care Spec Pharm 2017; 23: S5-S16.

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It’s very hard to explain a rare disease as IPF to your family and your friends because most people have never heard about it because it´s so rare.

– Dr. Marlies Wijsenbeek, Pulmonologist, The Netherlands