Mar 3, 2022

IPF: What is it?

Idiopathic pulmonary fibrosis (IPF) is an irreversible disease characterized by progressive loss of the ability of the lungs to effectively transport oxygen to the bloodstream due to scarring.¹ The cause is unknown.¹ With the advent of FDA-approved medicines and ongoing research there is new hope for people with IPF. There is no way to predict how long someone with IPF or other interstitial lung diseases (ILD) will live, but your doctor can give you more detailed information about your prognosis.¹ Check out this infographic and test your knowledge about IPF.

The most common symptoms of IPF are:

Shortness of breath¹

Persistent dry, hacking cough¹

Doctor can hear Velcro-like crackle in the lungs²

in the United States have IPF ³

IPF typically occurs in people

> 50
Years Old⁴

It tends to affect

More Men

than women⁴

Test your knowledge on what you just learned in the quiz below:

1. One of the most common symptoms of IPF is¹:

Coughing up thick mucus

Shortness of breath

Chest pain

Lightheadedness

Shortness of breath is one of the most common symptoms of IPF.

2. Approximately how many people in the U.S. are living with IPF?³

400,000

350,000

200,000

50,000

There are currently approximately 200,000 people in the U.S. living with IPF.

3. True or False? IPF typically affects people over 50 years old.⁴

False

True

IPF typically affects people over 50 years old.

IPF: Misdiagnosis is Common

IPF and other ILDs can share symptoms with other forms of respiratory and cardiovascular illness and can be misdiagnosed.⁵ Check out this infographic and take a short quiz about IPF and how commonly it is misdiagnosed.

~55%

of people with ILD, including IPF, reported at least one misdiagnosis,⁶

38%

reported two or more misdiagnoses⁶

~14% initially misdiagnosed with asthma⁶

~13% with pneumonia⁶

~12% with bronchitis⁶

~10% with allergies⁶

~7% with chronic obstructive pulmonary disease (COPD)⁶

72% of people with IPF or other ILDs reported that they initially attributed some or all of their respiratory symptoms to advancing age⁶

A person may see multiple doctors and specialists before receiving a correct diagnosis of IPF⁶

IPF is difficult to diagnose because:

Lung fibrosis can't always be seen on a regular chest X-ray⁷

Many other lung and heart diseases cause similar symptoms^8,9

People with IPF often have other co-morbidities, including those that may affect their breathing⁸

Test your knowledge on what you just learned in the quiz below:

1. True or false? Lung fibrosis can usually be seen on a regular chest X-ray.⁷

False

True

Lung fibrosis usually can’t be seen on a regular chest X-ray and requires a special scan called a high-resolution computed tomography scan (HRCT).

2. How many people with IPF or other ILDS were initially misdiagnosed?⁶

55 percent

25 percent

15 percent

5 percent

More than half of people with IPF are initially misdiagnosed with other forms of cardiovascular or respiratory illness, like COPD or asthma.

3. True or false? Most people are correctly diagnosed with IPF at initial doctor’s visit.⁶

False

True

Many patients are treated for several other conditions before a correct diagnosis of IPF is made.

IPF: Diagnosis and Disease Management

There is no cure for IPF, but a timely diagnosis is a critical factor for disease management since FDA-approved medicines have been shown to slow disease progression.¹⁰ Check out this infographic and test your knowledge about why diagnosis is so important.

68%

of people with ILD said consulting a respiratory specialist was the most important contributing factor in obtaining a clear diagnosis⁶

There is no cure for IPF, but a timely diagnosis is important for disease management; a longer delay in access to respiratory specialist care is associated with an increased risk of death¹⁰

Besides a lung transplant, there is unfortunately no cure for IPF at this time. However, there are disease management options for people with IPF, including:

FDA-approved Medications¹

Pulmonary rehabilitation¹

Lifestyle modifications (e.g., quit smoking)¹

Oxygen therapy¹

Ask your doctor for more information on lung transplantation and disease management options.

Test your knowledge on what you just learned in the quiz below:

1. Which of the following is not a recognized disease management option for people with IPF?¹

Lung transplantation

Lifestyle modification (e.g., quitting smoking)

Using a humidifier

Medicines approved specifically for IPF

While disease management strategies for IPF are highly individualized, lifestyle modifications, lung transplantation, and medicines approved for IPF are all possible options.

2. True or false? Quick diagnosis is important for disease management.¹⁰

True

False

Quick diagnosis is important for disease management.

¹ Pulmonary Fibrosis Foundation. Idiopathic Pulmonary Fibrosis. Available at: https://www.pulmonaryfibrosis.org/understanding-pff/types-of-pulmonary-fibrosis/idio pathic-pulmonary-fibrosis

² Cottin, et al. (2012). Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? European Respiratory Journal; 40: 519-521.

³ Pulmonary Fibrosis Foundation. Pulmonary Fibrosis Information Guide. Available at: https://www.pulmonaryfibrosis.org/docs/default-source/programs/educational-material s/pf-information-guides/pf-info-guide.pdf?sfvrsn=129c58c8_27.

⁴ American Lung Association. 7 Things Everyone Should Know about Pulmonary Fibrosis. Available at: https://www.lung.org/blog/7-things-know-pulmonary-fibrosis

⁵ Collard, et al. (2007). Patient Experiences with Pulmonary Fibrosis. Respiratory Medicine; 101: 1350-1354.

⁶ Cosgrove, et al. (2018). Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulmonary Medicine; 18:9.

⁷ Mayo Clinic. Pulmonary Fibrosis. Available at: https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/diagnosis-treatmen t/drc-20353695

⁸ Spagnolo, et al. (2012). Idiopathic Pulmonary Fibrosis: diagnostic pitfalls and therapeutic challenges. Multidisciplinary Respiratory Medicine; 7:42: 1-10.

⁹ Cleemput, et al. (2019). Idiopathic Pulmonary Fibrosis for Cardiologists: Differential Diagnosis, Cardiovascular Comorbidities, and Patient Management. Advances in Therapy; 36, 298–317.

¹⁰ Lamas, et, al. (2011). Delayed Access and Survival in IPF. American Journal of Respiratory and Critical Care Medicine; 184: 842-847.

Back to Respiratory Health

Respiratory Health

Understanding Idiopathic Pulmonary Fibrosis

IPF: What is it?

The most common symptoms of IPF are:

Shortness of breath¹

Persistent dry, hacking cough¹

Doctor can hear Velcro-like crackle in the lungs²

in the United States have IPF ³

IPF typically occurs in people

It tends to affect

than women⁴

Test your knowledge on what you just learned in the quiz below:

1. One of the most common symptoms of IPF is¹:

2. Approximately how many people in the U.S. are living with IPF?³

3. True or False? IPF typically affects people over 50 years old.⁴

IPF: Misdiagnosis is Common

of people with ILD, including IPF, reported at least one misdiagnosis,⁶

reported two or more misdiagnoses⁶

72% of people with IPF or other ILDs reported that they initially attributed some or all of their respiratory symptoms to advancing age⁶

A person may see multiple doctors and specialists before receiving a correct diagnosis of IPF⁶

IPF is difficult to diagnose because:

Lung fibrosis can't always be seen on a regular chest X-ray⁷

Many other lung and heart diseases cause similar symptoms^8,9

People with IPF often have other co-morbidities, including those that may affect their breathing⁸

Test your knowledge on what you just learned in the quiz below:

1. True or false? Lung fibrosis can usually be seen on a regular chest X-ray.⁷

2. How many people with IPF or other ILDS were initially misdiagnosed?⁶

3. True or false? Most people are correctly diagnosed with IPF at initial doctor’s visit.⁶

IPF: Diagnosis and Disease Management

of people with ILD said consulting a respiratory specialist was the most important contributing factor in obtaining a clear diagnosis⁶

There is no cure for IPF, but a timely diagnosis is important for disease management; a longer delay in access to respiratory specialist care is associated with an increased risk of death¹⁰

Besides a lung transplant, there is unfortunately no cure for IPF at this time. However, there are disease management options for people with IPF, including:

FDA-approved Medications¹

Pulmonary rehabilitation¹

Lifestyle modifications (e.g., quit smoking)¹

Oxygen therapy¹

Ask your doctor for more information on lung transplantation and disease management options.

Test your knowledge on what you just learned in the quiz below:

1. Which of the following is not a recognized disease management option for people with IPF?¹

2. True or false? Quick diagnosis is important for disease management.¹⁰

Tags

Understanding Idiopathic Pulmonary Fibrosis

IPF: What is it?

The most common symptoms of IPF are:

Shortness of breath1

Persistent dry, hacking cough1

Doctor can hear Velcro-like crackle in the lungs2

in the United States have IPF 3

IPF typically occurs in people

It tends to affect

than women4

Test your knowledge on what you just learned in the quiz below:

1. One of the most common symptoms of IPF is1:

2. Approximately how many people in the U.S. are living with IPF?3

3. True or False? IPF typically affects people over 50 years old.4

IPF: Misdiagnosis is Common

of people with ILD, including IPF, reported at least one misdiagnosis,6

reported two or more misdiagnoses6

72% of people with IPF or other ILDs reported that they initially attributed some or all of their respiratory symptoms to advancing age6

A person may see multiple doctors and specialists before receiving a correct diagnosis of IPF6

IPF is difficult to diagnose because:

Lung fibrosis can't always be seen on a regular chest X-ray7

Many other lung and heart diseases cause similar symptoms8,9

People with IPF often have other co-morbidities, including those that may affect their breathing8

Test your knowledge on what you just learned in the quiz below:

1. True or false? Lung fibrosis can usually be seen on a regular chest X-ray.7

2. How many people with IPF or other ILDS were initially misdiagnosed?6

3. True or false? Most people are correctly diagnosed with IPF at initial doctor’s visit.6

IPF: Diagnosis and Disease Management

of people with ILD said consulting a respiratory specialist was the most important contributing factor in obtaining a clear diagnosis6

There is no cure for IPF, but a timely diagnosis is important for disease management; a longer delay in access to respiratory specialist care is associated with an increased risk of death10

Besides a lung transplant, there is unfortunately no cure for IPF at this time. However, there are disease management options for people with IPF, including:

FDA-approved Medications1

Pulmonary rehabilitation1

Lifestyle modifications (e.g., quit smoking)1

Oxygen therapy1

Ask your doctor for more information on lung transplantation and disease management options.

Test your knowledge on what you just learned in the quiz below:

1. Which of the following is not a recognized disease management option for people with IPF?1

2. True or false? Quick diagnosis is important for disease management.10

Tags

Respiratory Health

Shortness of breath¹

Persistent dry, hacking cough¹

Doctor can hear Velcro-like crackle in the lungs²

in the United States have IPF ³

than women⁴

1. One of the most common symptoms of IPF is¹:

2. Approximately how many people in the U.S. are living with IPF?³

3. True or False? IPF typically affects people over 50 years old.⁴

of people with ILD, including IPF, reported at least one misdiagnosis,⁶

reported two or more misdiagnoses⁶

72% of people with IPF or other ILDs reported that they initially attributed some or all of their respiratory symptoms to advancing age⁶

A person may see multiple doctors and specialists before receiving a correct diagnosis of IPF⁶

Lung fibrosis can't always be seen on a regular chest X-ray⁷

Many other lung and heart diseases cause similar symptoms^8,9

People with IPF often have other co-morbidities, including those that may affect their breathing⁸

1. True or false? Lung fibrosis can usually be seen on a regular chest X-ray.⁷

2. How many people with IPF or other ILDS were initially misdiagnosed?⁶

3. True or false? Most people are correctly diagnosed with IPF at initial doctor’s visit.⁶

of people with ILD said consulting a respiratory specialist was the most important contributing factor in obtaining a clear diagnosis⁶

There is no cure for IPF, but a timely diagnosis is important for disease management; a longer delay in access to respiratory specialist care is associated with an increased risk of death¹⁰

FDA-approved Medications¹

Pulmonary rehabilitation¹

Lifestyle modifications (e.g., quit smoking)¹

Oxygen therapy¹

1. Which of the following is not a recognized disease management option for people with IPF?¹

2. True or false? Quick diagnosis is important for disease management.¹⁰