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What Is Idiopathic Pulmonary Fibrosis (IPF)?

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What Is IPF?

IPF is a specific type of interstitial lung disease (ILD), or diseases that cause inflammation and scarring of the lungs

Letter I Icon

The I stands for “idiopathic,” which means the cause of IPF is unknown.

Letter P Icon

“Pulmonary” is another word for lungs.

Letter F Icon

“Fibrosis” refers to scarring.

Know the characteristics of IPF

IPF is progressive. This means that the scarring caused by IPF will continue to happen, and lung function will continue to get worse. Overtime, scarring becomes more widespread and lung function declines. IPF progresses rapidly for some people and slowly for others.

IPF is unpredictable. It is difficult to know how slowly or quickly IPF will progress. Each person's experience is different.

IPF is irreversible. Once there is scarring in the lungs, it cannot be reversed. Today, there is no cure for IPF, but there are treatment options that may slow its progression.

Ask your doctor about the progression of IPF over time.

How IPF Affects the Lungs

IPF is scarring of tissue in your lungs

This scarring, or fibrosis, may be the result of tiny injuries deep in your lungs that do not heal properly.

These injuries lead to progressive scarring of tissue in the small air sacs in your lungs (called alveoli). This scarring, or fibrosis, causes the lungs to stiffen.

Fibrosis may damage the lungs’ ability to exchange gases, such as oxygen with the blood.

Did you know that the average lung function loss in adults without IPF is 30 mL/year? But in IPF, the progressive scarring in the lung can lead to much greater annual loss.

IPF permanently damages lung function

Currently, there is no cure for IPF, but there are treatment options that may help delay disease progression.

How Does IPF Affect Me?

With IPF, once damage occurs, it cannot be undone. That is why it is important to slow your IPF from progressing. The goal of treatment is to help slow progression of the disease.

Learn how Esbriet may help slow IPF

How Esbriet May Help Slow IPF

Preserving more of your lung function by slowing disease progression is essential, and Esbriet may help.

Select Important Safety Information and Indication

About Esbriet

Esbriet® (pirfenidone) is a prescription medicine used to treat people with a lung disease called idiopathic pulmonary fibrosis (IPF).

It is not known if Esbriet is safe and effective in children.

Before you take Esbriet, tell your doctor if you:
  • have other medical conditions (particularly liver or kidney problems).
  • are a smoker.
  • are or plan to become pregnant or breastfeed (Esbriet has not been studied in these patients).
  • are taking any prescription or over-the-counter medicines, vitamins, or herbal supplements.
What are the possible side effects of Esbriet?

Esbriet may cause serious side effects, including:

  • liver problems. Call your doctor if you have symptoms such as yellowing of your skin or eyes, dark or brown urine, pain on the upper right side of your stomach area, bleeding or bruising more easily than normal, or increased fatigue. Your doctor will also do regular blood tests to check your liver.
  • sun sensitivity and rash. When you are outside, use sunscreen (SPF 50) and wear a hat and clothes that cover your skin to avoid getting a sunburn.
  • severe skin reactions. Call your doctor right away if you have a severe skin reaction such as skin blisters, rash, sores in the mouth, hives or any other severe skin symptoms.
  • stomach problems. Esbriet may cause stomach problems such as nausea, vomiting, diarrhea, indigestion, heartburn, and stomach pain.

Your doctor may change your dose or discontinue Esbriet if side effects do not go away.

The most common side effects of Esbriet include upper respiratory tract infections, feeling tired, headache, dizziness, loss of appetite, sinusitis, insomnia, or weight loss.

These are not all the possible side effects of Esbriet.

What should you avoid while taking Esbriet?
  • Direct exposure to sunlight, or light from sunlamps and tanning beds.
  • Other medicines that can make your skin sensitive to sunlight.
  • Smoking, which may affect how well Esbriet works.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch or to Legacy Pharma Inc. SEZC at 1-800-727-7151.

Please see full Prescribing Information, including Patient Information, for additional important safety information.