Idiopathic Pulmonary Fibrosis (IPF)

What is idiopathic pulmonary fibrosis?

Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred over time. The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can't pinpoint the cause of the condition. When a cause can't be found, the condition is termed idiopathic pulmonary fibrosis (IPF). The formation of scar tissue is called fibrosis. This thickened, stiff tissue makes it more difficult for your lungs to work properly. In IPF, scarring begins in the air sac walls and the spaces around them, making the walls of the air sacs thicker. It is then harder for oxygen to pass through the air sac walls into the bloodstream. IPF is a serious disease that usually affects middle-aged and older adults. Many people live only about 3 to 5 years after diagnosis. The most common cause of death related to IPF is respiratory failure
Risk factors
  • Cigarette smoking
  • Viral infections, including Epstein-Barr virus (which causes mononucleosis), influenza A virus, hepatitis C virus, HIV, and herpes virus 6
  • Being male
  • Working around dust or fumes
IPF diagnosis is based on one’s medical history, a physical exam, and tests including:
  • Chest x-ray
  • High-resolution computed tomography
  • Lung function tests
  • Pulse oximetry
  • Arterial blood gas test
  • Skin test for tuberculosis
  • Exercise testing
  • Lung biopsy
  • Video-assisted thoracoscopy
  • Bronchoscopy
  • Bronchoalveolar lavage
  • Thoracotomy
Doctors may prescribe medicines, oxygen therapy, pulmonary rehabilitation (PR), and lung transplant to treat idiopathic pulmonary fibrosis.
  • 9/10 people with IPF also have gastroesophageal reflux disease (GERD).
  • 2/3 of patients are older than 60 at the time of diagnosis
IPF about

  • "What Is Idiopathic Pulmonary Fibrosis?" National Institutes of Health. U.S. Department of Health and Human Services, 20 Sept. 2011. Web. 11 May 2017.
  • "Idiopathic Pulmonary Fibrosis | IPF Symptoms & Treatment." Cleveland Clinic. Cleveland Clinic, n.d. Web. 11 May 2017.
  • "Pulmonary Fibrosis." Mayo Clinic. Mayo Foundation for Medical Education and Research, 23 Sept. 2016. Web. 11 May 2017.


In IPF, the lung tissue becomes scarred and can no longer function normally. As the long tissue becomes more heavily scarred, it becomes increasingly difficult to breathe and deliver oxygen to the rest of the body. Unfortunately, IPF is a disabling disease with no known cure, but there are a few treatment options. Check out this infographic to learn more about idiopathic pulmonary fibrosis:
idiopathic pulmonary fibrosis (IPF) about


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