Pulmonary Fibrosis

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.

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.

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.

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.

What causes pulmonary fibrosis?

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.

Who is more likely to get pulmonary fibrosis?

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:

• Older age: Most people who get pulmonary fibrosis develop it in the second half of life, between ages 50 and 70.
• Male biological sex: Pulmonary fibrosis affects more men than women. However, cases in women have risen in recent years.
• Smoking: Cigarette smoking increases your risk of getting pulmonary fibrosis.
• Working around dust or fumes: Regularly breathing in chemicals or hazardous substances can damage the lungs. Farmers, ranchers, hairdressers, stone cutters/polishers and metal workers may be at increased risk.
• Other medical conditions: In some cases, another medical condition (such as the autoimmune disease rheumatoid arthritis or a viral infection) leads to pulmonary fibrosis .
• Other factors: Radiation exposure, such as radiation therapy to treat cancer, can damage lung tissue. So can some medications, including chemotherapy and certain heart medications.

Can pulmonary fibrosis be hereditary?

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.

What are the symptoms of 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:

• Breathing in short, shallow spurts.
• Dry cough that doesn’t go away.
• Fatigue (extreme tiredness, no matter how much you sleep).
• Shortness of breath, especially during or soon after you exercise.
• Weight loss that’s not on purpose or easily explained.

As the disease progresses, some people experience:

• Clubbing, fingertips or toes that look different, such as wider or more round.
• Cyanosis, bluish skin (in fair-skinned people) or gray or white skin around the mouth or eyes (in dark-skinned people) from too little oxygen in the blood.

What does pulmonary fibrosis feel like?

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.

How is pulmonary fibrosis diagnosed?

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:

• Blood tests: Your provider may order blood tests to rule out other illnesses or reasons for your symptoms. Lab tests can also help providers track disease progression (how it affects your body over time) after diagnosis.
• Imaging tests: A chest X-ray or a CT scan may help rule out other lung-related illnesses. These pictures can clearly show lung scarring and may confirm a pulmonary fibrosis diagnosis.
• Breathing tests: These tests are also called pulmonary function tests. Different devices measure lung function and capacity (how well your lungs work).
• Oxygen desaturation study: This test measures oxygen levels in your blood. You walk for six minutes with a probe attached to your finger or forehead.
• Biopsy: A surgeon removes a small lung tissue sample through a small incision in the ribs. Providers sometimes perform a lung biopsy to confirm a pulmonary fibrosis diagnosis.

Can pulmonary fibrosis be reversed?

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.

How is pulmonary fibrosis treated?

Most pulmonary fibrosis treatments focus on easing symptoms and improving your quality of life.

Your provider may recommend one or more treatments:

• Medication: Two medications — pirfenidone (Esbriet®) and nintedanib (OFEV®) —may slow down lung scarring. These medications can help preserve lung function.
• Oxygen therapy: Giving your body extra oxygen helps you breathe more easily. It may also increase your energy and strength.
• Pulmonary rehabilitation: Staying active in this special exercise program may improve how much (or how easily) you can do everyday tasks or activities.
• Lung transplant: A lung transplant replaces one or both diseased lungs with a healthy lung (or lungs) from a donor. It offers the potential to improve your health and quality of life. A lung transplant is major surgery, and not everyone is a candidate. Ask your provider if you may be eligible for a lung transplant.

Can pulmonary fibrosis be cured?

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.

What are the complications of pulmonary fibrosis?

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.

Can I prevent pulmonary fibrosis?

Unfortunately, you can’t prevent pulmonary fibrosis.

How does pulmonary fibrosis progress?

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).

What is the life expectancy for people who have pulmonary fibrosis?

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:

• Be proactive to avoid getting sick: Pay close attention to handwashing, and avoid direct contact with anyone you know is sick. Having pulmonary fibrosis means your body can’t recover as easily from infections that other people may fight off with little effort.
• Keep up-to-date with vaccines: Lung scarring makes it harder for the body to fight against infections. Get vaccinated for pneumonia and influenza (flu) to minimize your risk.
• Practice healthy habits: Stay active and make smart food choices. Get plenty of rest, and quit smoking.

When should I call my provider?

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.