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.
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.
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 information about lung function, go to the Health Topics How the Lungs Work article.
Clinical trials are research studies that explore whether a medical strategy, treatment, or device is safe and effective for humans. To find clinical trials that are currently underway for Idiopathic Pulmonary Fibrosis, visit www.clinicaltrials.gov.
May 19, 2014
Common treatment for patients with chronic, progressive lung disease found to be ineffective
A drug used to treat patients with mild to moderate lung damage from the disease idiopathic pulmonary fibrosis (IPF) is no better than placebo for preserving lung function, according to a study supported by the National Institutes of Health. The finding is in the final report of a clinical trial called Prednisone, Azathioprine, and N-Acetylcysteine: A Study That Evaluates Response in Idiopathic Pulmonary Fibrosis (PANTHER-IPF).
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