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Idiopathic Pulmonary Fibrosis

What Is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic pulmonary fibrosis (IPF) causes scar tissue to grow inside your lungs. Usually, when you breathe in, oxygen moves through tiny air sacs into your bloodstream. From there, it travels to organs in your body.

IPF scar tissue is thick, like the scars you get on your skin after a cut. It slows oxygen flow from your lungs to your blood, which can keep your body from working as it should. Low oxygen levels and the stiff scar tissue make it hard to breathe.

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There’s no cure for IPF. The illness will have an impact on your life and your family. For most people, symptoms don’t get better, but there are new treatments that can slow the damage to your lungs. Everyone’s outlook is different. Some people will get worse quickly, while others can live 10 years or more after they are diagnosed. There are therapies to help you breathe easier and manage your symptoms. In some cases you may be able to have a lung transplant.

Causes

Some people get pulmonary fibrosis when they're exposed to something in their environment, like pollution, certain medicines, or an infection. But most of the time, doctors don't know what causes IPF. That's what "idiopathic" means.

You might be more likely to get IPF if you:

  • Breathe in wood or metal dust at work or home
  • Have acid reflux disease
  • Smoke cigarettes

Sometimes, IPF runs in families. Doctors think broken genes could cause the disease in some people. No one knows yet which specific genes may be involved.

Symptoms

You can have IPF for a long time without noticing any symptoms. After many years, the scarring in your lungs gets worse, and you might have:

  • A dry, hacking cough that doesn't go away
  • Shortness of breath, especially when you walk or do other activities

You might also notice that:

  • You feel more tired than usual
  • Your joints and muscles ache
  • You've lost weight without trying
  • The tips of your fingers and toes have gotten wider, called clubbing
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