Idiopathic Pulmonary Fibrosis, Explained in 90 Seconds

Idiopathic pulmonary fibrosis gets its name from an intimidating blend of ancient Greek and Latin, so let’s break this down:

Idiopathic: “Of unknown cause”

Pulmonary: “Affecting the lungs”

Fibrosis: “Scarring”

In other words, idiopathic pulmonary fibrosis, or IPF, is a chronic lung condition in which lung tissue becomes scarred—and doctors don’t know what causes it.

How IPF affects lung function

In normal, healthy lungs, your body exchanges oxygen and carbon dioxide within the alveoli. These are clusters of tiny air sacs, and your lungs have millions of them. The alveoli sit at the end of the bronchioles, or the branches of airways inside the lungs. Thanks to the alveoli, your blood gets repeatedly oxygenated. This allows your organs and other tissue to get the nutrients they need to function well.

In someone with IPF, the soft and sponge-like lung tissue thickens and stiffens. Scar tissue forms around and between alveoli, reducing their ability to exchange oxygen and carbon dioxide.

Symptoms of IPF progress over time. They include shortness of breath and cough, but symptoms may vary from patient to patient. For some reason, some patients progress rapidly, while others may remain steady for long periods of time. (Here are other possible causes of shortness of breath.)

If it worsens, complications of IPF include:

• Pulmonary hypertension: This refers to high blood pressure in the arteries connecting the heart and lungs. This may result in poor blood flow.

• Respiratory failure: This is when the body lacks adequate oxygen (hypoxia), which causes shortness of breath, wheezing, and fatigue.

Living with IPF

Doctors do not yet have a cure for IPF. Still, treatment may help reduce symptoms, slow the progression, and improve quality of life.

Medications are available to help slow the rate of scarring and reduce inflammation. This can help retain lung function and reduce the risk of complications.

For some, long-term oxygen therapy may be necessary. This treatment uses devices that deliver concentrated oxygen through a mask or nasal cannula (a flexible tube that is attached via prongs into the nostrils). Oxygen therapy may help reduce symptoms of IPF and improve your quality of life.

While the cause of IPF isn’t known, people who smoke tend to have a higher risk. For help quitting smoking for good, or if you’re experiencing shortness of breath and don’t know why, talk to your doctor.