Pulmonary fibrosis is a serious, lifelong lung disease. It causes lung scarring (tissues scar and thicken over time), making it harder to breathe. Symptoms may come on quickly or take years to develop. No cure exists. Medications may slow down scarring and help preserve lung function. Oxygen therapy and staying active may relieve symptoms.
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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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.
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.
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:
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.
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.
Many things (like smoking) can lead to pulmonary fibrosis. Often, the cause remains unknown (idiopathic pulmonary fibrosis). Some types of pulmonary fibrosis may run in families.
Certain risk factors, like smoking, may make it more likely you could develop pulmonary fibrosis. But even having one or more risk factors doesn't mean you’ll definitely get the disease one day.
Other risk factors of pulmonary fibrosis include:
Medical experts do believe people can inherit this disease through genes that run in families. However, inheriting pulmonary fibrosis is very rare. Researchers still have much to learn about how (and which) genes may cause pulmonary fibrosis.
Pulmonary fibrosis doesn’t affect everyone in the same way. Many common, easily treatable conditions can cause similar symptoms. Sometimes these symptoms are signs of the common cold or an upper respiratory infection.
Pulmonary fibrosis symptoms include:
As the disease progresses, some people experience:
Lung scarring due to pulmonary fibrosis makes it harder to breathe. You may feel like you can’t catch your breath or breathe deeply, no matter how hard you try.
Your healthcare provider will ask you about your medical history. You’ll also have a physical exam to carefully evaluate your symptoms. They may use a stethoscope to listen to you breathe, listening for abnormal sounds (like crackling).
Pulmonary fibrosis can look a lot like other, more common lung diseases, which can make diagnosis challenging. Your provider may also order one or more tests to diagnose pulmonary fibrosis:
Unfortunately, lung damage due to pulmonary fibrosis is permanent (not reversible). Getting diagnosed and starting treatment as early as possible may help your lungs work better, longer.
Most pulmonary fibrosis treatments focus on easing symptoms and improving your quality of life.
Your provider may recommend one or more treatments:
No cure for pulmonary fibrosis exists today. But researchers around the world are working to change that.
Ask your provider if you might be eligible for a clinical trial. Participating in an ongoing trial may allow you to try one of the newest pulmonary fibrosis treatment options.
Scarred lung tissues have a hard time getting oxygen to the rest of the body. This strains the right side of the heart. It may lead to high blood pressure in the lungs (called pulmonary hypertension). In severe cases, it can cause heart failure.
Unfortunately, you can’t prevent pulmonary fibrosis.
Pulmonary fibrosis symptoms typically develop slowly. You may notice vague signs (or no symptoms at all) in the early stages. How long it takes for symptoms to get worse is different for everyone.
Healthcare providers can’t easily predict how pulmonary fibrosis progresses. Your symptoms may get worse very slowly, over years. In some cases, the disease may lead to severe symptoms rather quickly (over months).
Some people live only months after a pulmonary fibrosis diagnosis. Others live several years. Many factors affect your prognosis. Even your provider can’t predict some of these factors.
If you’ve been diagnosed with pulmonary fibrosis, you can take steps to help your body stay in its best possible shape:
In some cases, pulmonary fibrosis gets a lot worse, all at once. You should call your provider if you suddenly feel a big difference in symptoms.
For example, call your provider if you’re coughing a lot more than usual or have any other symptoms that seem new or alarming. See a provider right away if you experience any difficulty breathing that you can’t control.
A note from Cleveland Clinic
If you have pulmonary fibrosis, you will likely see a pulmonologist (lung specialist) long-term for regular follow-up visits. Your healthcare provider will work with you to preserve your lung function and maintain the highest possible quality of life. They will help find the right combination of medication, supportive care and clinical trials to treat the ways pulmonary fibrosis affects you. Connecting with a pulmonary fibrosis support group may provide firsthand insights and advice from people who have faced similar challenges.
Last reviewed by a Cleveland Clinic medical professional on 04/05/2021.
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