What is pulmonary fibrosis?
Pulmonary fibrosis is a group of serious lung diseases that affect the respiratory system. Pulmonary fibrosis scars and thickens lung tissue. It impacts the connecting tissue in the lung and the alveoli (air sacs inside the lungs).
The lung damage gradually gets worse over time. Hard, stiff lung tissues don’t expand as well as they should, making it harder to breathe. Pulmonary fibrosis may cause shortness of breath when you do routine tasks that never seemed tiring before.
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What are alveoli?
Alveoli are tiny, delicate air sacs in your lungs. They help get oxygen into the bloodstream when you inhale.
In pulmonary fibrosis, the thin walls of these air sacs start to scar and thicken. When that happens, it’s harder for the air sacs to do their job and get oxygen to the rest of the body.
Is pulmonary fibrosis a terminal illness?
Yes, healthcare providers typically consider pulmonary fibrosis a terminal illness. Pulmonary fibrosis is a progressive disease (gets worse over time). There is no cure, and it eventually leads to death.
Many things factor into how long and well people can live with pulmonary fibrosis. The disease may get worse quickly (over months) or very slowly (over years). Newer medications may help slow the disease progression. Research continues to focus on improving therapies.
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Are pulmonary fibrosis and COPD the same?
No, pulmonary fibrosis and chronic obstructive pulmonary disease (COPD) are not the same. However, they are similar in some ways. Pulmonary fibrosis and COPD are both lung diseases that get worse over time. Both conditions can make breathing difficult.
But these conditions affect your lungs differently:
- Pulmonary fibrosis: Providers classify pulmonary fibrosis as an interstitial lung disease. The interstitial tissues are cells that make up the space between blood vessels and other structures inside the lungs. Pulmonary fibrosis damages these cells. It is a rare disease.
- COPD: COPD is a more common type of lung disease. Diseases like emphysema and chronic bronchitis are types of COPD. In COPD, lung tissue is damaged, alveoli are destroyed and airways can get irritated and inflamed (swollen).
What is idiopathic pulmonary fibrosis?
Idiopathic is a term providers use when they can’t determine what caused a condition. Idiopathic pulmonary fibrosis is the most common type of interstitial lung disease.
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How common is pulmonary fibrosis?
Medical experts have a hard time pinpointing exactly how many people have pulmonary fibrosis. According to one study, idiopathic pulmonary fibrosis affects at least 200,000 people in the U.S.
