InterMune Know IPF Now

InterMune Know IPF Now

What is IPF?

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease that causes permanent damage to the lungs. It may be helpful to understand some common terms your doctor may use:

Interstitial lung disease (ILD) describes a large group of lung disorders that cause inflammation and/or scarring (fibrosis) of lung tissue.

Pulmonary fibrosis occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. The scarring associated with pulmonary fibrosis can be caused by a multitude of factors.

Idiopathic pulmonary fibrosis is an interstitial lung disease that results in scarring or fibrosis of the lungs for unknown reasons. It is one of the most common interstitial lung diseases.

The tissue damage that occurs with IPF may be the result of abnormal healing in response to tiny injuries in the deepest tissues within the lungs. In response to these injuries, tiny air sacs in the lung called alveoli (al-VEE-oh-lie) thicken and stiffen, causing a type of scarring (fibrosis). This makes it difficult for the oxygen you breathe in to enter the bloodstream.

  • Interactive background.
    • Interactive alveoli.
    • Interactive scarring and fibrosis.
  • IPF lung demonstrating thickening and stiffening of the alveoli, causing fibrosis. As IPF progresses, continued fibrosis reduces the ability of the lungs to move oxygen into the bloodstream

Early in the disease, there is normal tissue next to scarred tissue. As the disease progresses, fibrosis becomes more widespread. Over time, continued fibrosis reduces the ability of the lungs to move oxygen into the bloodstream. Currently, there is no treatment to repair the damage to lung tissue caused by IPF.

IPF: a disease with an unknown cause

Unlike other forms of interstitial lung disease, 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 others with similar risk factors do not.

While 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.

Potential risk factors

There are no proven causes of IPF. However, certain factors, such as smoking cigarettes, may increase the risk of developing IPF.
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  • Cigarette smoking: A history of smoking is strongly associated with IPF, especially in people who have smoked heavily or who have smoked for many years. However, there are also patients with IPF who have never smoked.

  • Gastroesophageal reflux disease (GERD): Some scientists believe that this digestive system disorder may cause tiny, repeated injuries to the lung tissue.

  • Infection: A large number of studies have examined the relationship between infection and IPF, but the findings are not conclusive.

  • Environmental pollutants: Exposure to airborne particles from metal or woodworking, farming, raising birds, stone cutting/polishing, livestock, or agricultural dust may increase the risk of IPF.

Who has IPF?