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Understanding Idiopathic Pulmonary Fibrosis


Back To Respiratory Health
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 diagnosis1,5,6:

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:

Shortness of breath
Lightheadedness
Chest pain
Coughing up thick mucus
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

True
False
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

50,000
100,000
200,000
400,000
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

True
False
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

True
False
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

5 percent
15 percent
25 percent
More than 50 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

True
False
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

1.5 years
2.2 years
3 years
4 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

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.

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

True
False
Quick diagnosis is important for disease management.

You Scored:

out of

Take It Again
  •   References Down arrow

    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


Tags:

  • Rare Disease,
  • Respiratory Health

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