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Idiopathic Pulmonary Fibrosis (IPF)

What is IPF?

Idiopathic pulmonary fibrosis (IPF) is an irreversible,1 unpredictable and fatal disease2 that makes breathing difficult and causes permanent damage to the lungs. With IPF, progressive scarring, or “fibrosis” of the lungs, prevents muscles, the heart and other organs from receiving enough oxygen to work properly.1 Eventually the lungs become hardened due to the fibrosis and stop working.1

How common is IPF?

IPF typically occurs in people older than 50, affecting slightly more men than women.3 Approximately 100,0004 people in the United States are affected by IPF.

What are the causes of IPF?

Unlike other forms of interstitial lung disease (which also cause scarring in the spaces between the air sacs in the lungs1), a diagnosis of IPF means that the cause of the lung scarring can’t be identified.

The medical community has not yet determined why some people develop IPF, while other people with similar risk factors do not. While the genetic factors in the disease are not well understood, scientists believe that, in some cases, IPF may be the result of an interaction between the environment and a person’s genetic (inherited) potential for the disease.

What are the most common symptoms?

Early signs and symptoms of IPF may include the following:1

  • A persistent cough that does not produce any phlegm
  • Shortness of breath, sometimes even during normal daily activities1,5
  • A doctor hears "Velcro®-like" crackles deep in the lungs

How is IPF diagnosed?

IPF is often initially misdiagnosed as other lung diseases,6 as its symptoms are similar to other respiratory diseases like chronic obstructive pulmonary disease. It can take months or even years to accurately diagnose IPF.

The medical community has not yet determined why some people develop IPF, while others with similar risk factors do not. Disease progression is unpredictable. IPF can progress quickly or slowly, and every patient is different. Preserving lung function is the ultimate goal of treating patients with IPF.

What is the purpose of pulmonary function tests?7

Pulmonary function tests help doctors assess how fibrosis is affecting a patient's lungs. Regular testing creates an important record of the health of the patient's lungs.

Through regular monitoring, doctors can determine how well a patient is responding to therapies to manage symptoms, how rapidly the condition is progressing, and potential next steps.

What happens as IPF progresses?

Most people with IPF experience shortness of breath and activities such as walking or climbing stairs may become more difficult over time. Shortness of breath occurs because lung scarring limits the amount of oxygen that can enter the bloodstream. As a result, muscles, the heart, and other organs don’t get the oxygen they need to function properly.

Over time, symptoms due to the lack of oxygen in the bloodstream may increase or become more severe, including:

  • Shortness of breath, even at rest
  • A dry cough that may become severe or pronounced, and bouts of coughing that cannot be controlled
  • Easily tiring, even during light activities
  • Clubbing (enlargement) of fingers that may develop over time
  • The need for supplemental oxygen therapy due to decreased oxygen saturation of the blood1

What is the life expectancy for people with IPF?

The median survival time from diagnosis is two to four years, with a five-year survival rate of approximately 30-50 percent,8 which makes IPF more rapidly lethal than many malignancies, including breast, ovarian and colorectal cancers.9

How is IPF treated?

Doctors may provide prescription drugs, supplements, oxygen, or over-the-counter medications to help manage the symptoms of IPF.10 Preservation of lung function is the ultimate goal of treating patients with IPF.11