Understanding Idiopathic Pulmonary Fibrosis

May 10, 2017

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.^1,2 With the advent of FDA-approved medicines and ongoing research there is new hope for people with IPF. The cause is unknown.² Check out this infographic and take a short quiz about IPF.

Approximate Five-year survival rate³:

30%

The most common symptoms of IPF are:

Shortness of breath²

Persistent dry, hacking cough²

Doctor can hear Velcro-like crackle in the lungs⁴

Median survival time from diagnosis^1,5,6:

3-5
Years

in the United States have IPF¹

IPF typically occurs in people

> 50
Years Old⁷

It tends to affect

More Men

than women⁷

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

1. One of the most common symptoms of IPF is²:

Coughing up thick mucus

Shortness of breath

Chest pain

Lightheadedness

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

2. True or false? The median survival time of people with IPF is 6 years.^1,5,6

False

True

The median survival after diagnosis with IPF is 3-5 years.

3. Approximately how many people in the U.S. are living with IPF?¹

400,000

200,000

100,000

50,000

There are currently approximately 100,000 people in the U.S. living with IPF.

4. True or False? IPF typically affects people over 50 years old.⁷

False

True

IPF typically affects people over 50 years old.

IPF: Misdiagnosis is Common

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.^1,2 IPF can share symptoms with other forms of respiratory and cardiovascular illness and is frequently misdiagnosed.⁸ Check out this infographic and take a short quiz about IPF and how commonly it is misdiagnosed.

More
than
50%

of people with IPF are initially misdiagnosed with other forms of respiratory or cardiovascular illness⁹

19% initially misdiagnosed with bronchitis⁹

14% with asthma⁹

8% with COPD⁹

7% with emphysema⁹

5% with heart disease⁹

55% of people with IPF reported at least a 1-year delay between early symptoms and diagnosis with IPF⁹

A person may see multiple doctors and specialists before receiving a correct diagnosis of IPF⁹

IPF is difficult to diagnose because:

Lung fibrosis usually can't be seen on a regular chest X-ray¹⁰

Many other lung and heart diseases cause similar symptoms¹¹

People with IPF often have other co-morbidities, including those that may affect their breathing¹¹

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.¹⁰

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 are initially misdiagnosed with other forms of respiratory or cardiovascular illness?⁹

More than 50 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.⁹

False

True

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

IPF: Diagnosis

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.^1,2 There is no cure for IPF, but quick diagnosis is a critical factor for disease management.¹² Check out this infographic and take a short quiz about IPF and why quick diagnosis is so important.

The median delay in access to specialist care is
2.2
years¹³

Compared to people receiving specialist care within a year of symptoms¹³...

There is no cure for IPF, but quick diagnosis is important for disease management.¹²

There are disease management options for people with IPF, including:

Pulmonary rehabilitation¹¹

Lifestyle modifications (e.g., quit smoking)¹¹

Oxygen therapy¹¹

Lung transplantation¹¹

Medications²

Ask your doctor for more information on these disease management options.

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

1. What is the median delay in access to specialist care in people with IPF?¹³

4 years

3 years

2.2 years

1.5 years

People with IPF have a median delay in access to specialist care of 2.2 years.

2. Which of the following is not a recognized disease management option for people with IPF?^2,11

Medicines approved specifically for IPF

Using a humidifier

Lifestyle modification (e.g., quitting smoking)

Lung transplantation

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

3. True or false? Quick diagnosis is important for disease management.¹²

False

True

Quick diagnosis is important for disease management.

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References

¹ Kistler, et al. (2014). Lung Transplantation in Idiopathic Pulmonary Fibrosis: A Systemic Review of the Literature. BMC Pulmonary Medicine.

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

³ National Institutes of Health. Idiopathic Pulmonary Fibrosis. Available at: http://ghr.nlm.nih.gov/condition/idiopathic-pulmonary-fibrosis.

⁴ Gribbin, et al. (2006). Incidence and Mortality of Idiopathic Pulmonary Fibrosis. Thorax.61: 980–985

⁵ Lee, et al. (2014). The burden of idiopathic pulmonary fibrosis: An unmet public health need. Respiratory Medicine; 108: 955-967.

⁶ American Thoracic Society (ATS) and European Respiratory Society (ERS). (2002). ATS Classification of the Idiopathic Interstitial Pneumonias. American Journal of Respiratory and Critical Care Medicine; 165: 277-304.

⁷ Coalition for Pulmonary Fibrosis (CPF). (2004). New Research on Idiopathic Pulmonary Fibrosis Suggests That 55 Percent of IPF Patients May Be Initially Misdiagnosed CPF Action Alert; April/May/June: 1-12.

⁸ Collard, et al. (2007). Patient Experiences with Pulmonary. Fibrosis Respiratory Medicine; 101: 1350-1354.

⁹ Collard, et al. (2015). Overview of Idiopathic Interstitial Pneumonias. Merck Manuals.

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

¹¹ Cottin, et al. (2014). Neglected Evidence in IPF and the Importance of Early Diagnosis and Treatment. European Respiratory Review; 23: 106-110.

¹² Lamas, et, al. (2011). Delayed Access and Survival in IPF.American Journal of Respiratory and Critical Care Medicine; 184: 842-847.

¹³ Raghu, et al., on behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. (2011). An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. American Thoracic Society Documents; 183: 788-824.

ESB/112415/0519(2) 04/18

Understanding Idiopathic Pulmonary Fibrosis

IPF: What is it?

Approximate Five-year survival rate3:

The most common symptoms of IPF are:

Shortness of breath2

Persistent dry, hacking cough2

Doctor can hear Velcro-like crackle in the lungs4

Median survival time from diagnosis1,5,6:

in the United States have IPF1

IPF typically occurs in people

It tends to affect

than women7

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

1. One of the most common symptoms of IPF is2:

2. True or false? The median survival time of people with IPF is 6 years.1,5,6

3. Approximately how many people in the U.S. are living with IPF?1

4. True or False? IPF typically affects people over 50 years old.7

IPF: Misdiagnosis is Common

of people with IPF are initially misdiagnosed with other forms of respiratory or cardiovascular illness9

55% of people with IPF reported at least a 1-year delay between early symptoms and diagnosis with IPF9

A person may see multiple doctors and specialists before receiving a correct diagnosis of IPF9

IPF is difficult to diagnose because:

Lung fibrosis usually can't be seen on a regular chest X-ray10

Many other lung and heart diseases cause similar symptoms11

People with IPF often have other co-morbidities, including those that may affect their breathing11

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.10

2. How many people with IPF are initially misdiagnosed with other forms of respiratory or cardiovascular illness?9

3. True or false? Most people are correctly diagnosed with IPF at initial doctor’s visit.9

IPF: Diagnosis

Compared to people receiving specialist care within a year of symptoms13...

There is no cure for IPF, but quick diagnosis is important for disease management.12

There are disease management options for people with IPF, including:

Pulmonary rehabilitation11

Lifestyle modifications (e.g., quit smoking)11

Oxygen therapy11

Lung transplantation11

Medications2

Ask your doctor for more information on these disease management options.

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

1. What is the median delay in access to specialist care in people with IPF?13

2. Which of the following is not a recognized disease management option for people with IPF?2,11

3. True or false? Quick diagnosis is important for disease management.12

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Respiratory Health

Approximate Five-year survival rate³:

Shortness of breath²

Persistent dry, hacking cough²

Doctor can hear Velcro-like crackle in the lungs⁴

Median survival time from diagnosis^1,5,6:

in the United States have IPF¹

than women⁷

1. One of the most common symptoms of IPF is²:

2. True or false? The median survival time of people with IPF is 6 years.^1,5,6

3. Approximately how many people in the U.S. are living with IPF?¹

4. True or False? IPF typically affects people over 50 years old.⁷

of people with IPF are initially misdiagnosed with other forms of respiratory or cardiovascular illness⁹

55% of people with IPF reported at least a 1-year delay between early symptoms and diagnosis with IPF⁹

A person may see multiple doctors and specialists before receiving a correct diagnosis of IPF⁹

Lung fibrosis usually can't be seen on a regular chest X-ray¹⁰

Many other lung and heart diseases cause similar symptoms¹¹

People with IPF often have other co-morbidities, including those that may affect their breathing¹¹

1. True or false? Lung fibrosis can usually be seen on a regular chest X-ray.¹⁰

2. How many people with IPF are initially misdiagnosed with other forms of respiratory or cardiovascular illness?⁹

3. True or false? Most people are correctly diagnosed with IPF at initial doctor’s visit.⁹

Compared to people receiving specialist care within a year of symptoms¹³...

There is no cure for IPF, but quick diagnosis is important for disease management.¹²

Pulmonary rehabilitation¹¹

Lifestyle modifications (e.g., quit smoking)¹¹

Oxygen therapy¹¹

Lung transplantation¹¹

Medications²

1. What is the median delay in access to specialist care in people with IPF?¹³

2. Which of the following is not a recognized disease management option for people with IPF?^2,11

3. True or false? Quick diagnosis is important for disease management.¹²