Ever heard of IPF, the lung disease that could be behind the clubbing of your fingertips?

.

What Is Idiopathic Pulmonary Fibrosis?

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


As the lung tissue thickens, your lungs can't properly move oxygen into your bloodstream. As a result, your brain and other organs don't get the oxygen they need. (For more information, go to the "How the Lungs Work" section of this article, the link is further down.)

Sometimes doctors can find out what's causing 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 disease that usually affects middle-aged and older adults. IPF varies from person to person. In some people, fibrosis happens quickly. In others, the process is much slower. In some people, the disease 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 of death include pulmonary hypertension (HI-per-TEN-shun), heart failure, pulmonary embolism (EM-bo-lizm), pneumonia (nu-MO-ne-ah), and lung cancer.

Genetics may play a role in causing IPF. If more than one member of your family has IPF, the disease is called familial IPF.

Research has helped doctors learn more about IPF. As a result, they can more quickly diagnose the disease now than in the past. Also, researchers are studying several medicines that may slow the progress of IPF. These efforts may improve the lifespan.


How the Lungs Work

To understand idiopathic pulmonary fibrosis (IPF), it helps to understand 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 bronchial (BRONG-ke-al) tubes or airways.

Figure A shows the location of the lungs and airways in the body. The inset image shows a detailed view of the lung's airways and air sacs in cross-section. Figure B shows fibrosis (scarring) in the lungs. The inset image shows a detailed view of the fibrosis and how it damages the airways and air sacs.

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 (al-VEE-uhl-eye). 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 pumps the oxygen-rich blood to all your body's organs. These organs can't function without an ongoing supply of oxygen.

The animation below shows how the lungs work. Click the "start" button to play the animation. Written and spoken explanations are provided with each frame. Use the buttons in the lower right corner to pause, restart, or replay the animation, or use the scroll bar below the buttons to move through the frames.

The animation shows how the lungs inhale oxygen and transfer it to the blood. It also shows how carbon dioxide (a waste product) is removed from the blood and exhaled.

In IPF, scarring begins in the air sac walls and the spaces around them. The scarring makes the walls of the air sacs thicker. This makes it harder for oxygen to pass through the air sac walls into the bloodstream.

For more details on causes and treatment