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What is Idiopathic Pulmonary Fibrosis?

By  ,  National Institute of Health
Jan 10, 2013
4.8 / 5(4 Ratings)

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.
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.


How the Lungs Work


The air that you breathe in through your nose or mouth travels down through your trachea (windpipe) into two tubes in your lungs called the bronchial tubes, or airways.
The airways are shaped like an upside-down tree with many branches. The windpipe is the trunk. It splits into two bronchial tubes, or bronchi. Thinner tubes called bronchioles branch out from the bronchi.

 

The bronchioles end in tiny air sacs called alveoli. These air sacs have very thin walls, and small blood vessels called capillaries run through them. There are about 300 million alveoli in a normal lung.


When the air that you've just breathed in reaches these air sacs, the oxygen in the air passes through the air sac walls into the blood in the capillaries. At the same time, carbon dioxide (a waste gas) moves from the capillaries into the air sacs. This process is called gas exchange.


The oxygen-rich blood in the capillaries then flows into larger veins, which carry it to the heart. Your heart then pumps the oxygen-rich blood to all your body's organs. These organs can't function without an ongoing supply of oxygen.


In idiopathic pulmonary fibrosis, scarring begins in the air sac walls and the spaces around them. It makes the walls of the air sacs thicker. As a result, oxygen can't move from the air sacs into the bloodstream.

 

Figure A shows...

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