What Is Idiopathic Pulmonary Fibrosis?

Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a condition in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The development of the scarred tissue is called fibrosis.

As the lung tissue becomes thicker, your lungs lose their ability to move oxygen into your bloodstream. As a result, your brain and other organs don't get the oxygen they need. (See the section on How the Lungs Work for more information.)

In some cases, doctors can find out what's causing the fibrosis. But in most cases, they can't find a cause. They call these cases idiopathic (id-ee-o-PATH-ick) pulmonary fibrosis (IPF).

IPF is a serious condition. About 200,000 Americans have it. About 50,000 new cases are diagnosed each year. IPF mostly affects people who are 50 to 75 years of age.

IPF varies from person to person. In some people, the lung tissue quickly becomes thick and stiff. In others, the process is much slower. In some people, the condition stays the same for years.

IPF has no cure yet. Many people live only about 3 to 5 years after diagnosis. The most common cause of death related to IPF is respiratory failure. Other causes include:

• Pulmonary hypertension
• Heart failure
• Pulmonary embolism (EM-bo-lizm)
• Pneumonia (nu-MO-ne-ah)
• Lung cancer

Some people may be more likely to develop IPF because of their genes (the basic units of heredity). If more than one member of your family has IPF, the condition is called familial idiopathic pulmonary fibrosis.

Today, scientists are beginning to understand more about what causes IPF, and they can diagnose it more quickly. They also are studying several medicines that may slow the progress of the disease. These efforts should improve the lifespan and quality of life for people who have IPF.

Revised May 2009