Pulmonary Fibrosis: What You Should Know About Stages and Life Expectancy

Pulmonary fibrosis is a chronic lung condition that causes dry cough and trouble breathing. If you or someone you know has been diagnosed with pulmonary fibrosis, you may have questions about what to expect in the future. This isn’t straightforward. But here is what research can offer about pulmonary fibrosis and life expectancy.

What is pulmonary fibrosis?

Pulmonary fibrosis is a condition that causes scar tissue to form in the lungs. Over time, fibrosis of the lungs makes it harder for them to expand and fill up with air. It also becomes more difficult for oxygen to move from the lungs into the bloodstream. This leads to symptoms like: 

• Dry cough that doesn’t go away

• Trouble breathing, especially with movement or activity

• Fast, shallow breathing

• Tiredness (fatigue)

• Nail changes (nail clubbing)

What’s the average pulmonary fibrosis life expectancy?

The answer isn’t simple. Without treatment, the average life expectancy for patients with pulmonary fibrosis is 3 to 5 years. But that number changes depending on a person’s specific situation. It can be hard for healthcare providers to offer someone concrete information on their life expectancy. 

That’s because not all pulmonary fibrosis behaves the same way over time. And some people find out they have pulmonary fibrosis when the condition is more advanced. Both of these characteristics impact a person’s long-term outlook (prognosis) and life expectancy. 

Also, people with pulmonary fibrosis can live longer if they get the right treatment. Each person with pulmonary fibrosis responds to treatment differently. How well someone responds to treatment will impact their prognosis too. 

What factors affect a person’s life expectancy with pulmonary fibrosis?

Many different factors affect the life expectancy of a person with pulmonary fibrosis. Here’s how each factor affects a person’s long-term outlook.

1. Type of pulmonary fibrosis 

People can develop pulmonary fibrosis for different reasons. Some things that can lead to pulmonary fibrosis include:

• Environmental exposures: Exposure to certain things like asbestos, silica, and molds can lead to pulmonary fibrosis. 

• Medications: Long-term exposure to certain medications can also lead to pulmonary fibrosis, including medications to treat cancer and autoimmune conditions. 

• Medical conditions: Certain autoimmune conditions, like lupus and rheumatoid arthritis, can also increase a person’s chance of developing pulmonary fibrosis. 

• Genetics: Pulmonary fibrosis can run in families too. 

Many times, it’s not clear why someone develops pulmonary fibrosis. In these cases, people are diagnosed with “idiopathic pulmonary fibrosis.”

Different types of pulmonary fibrosis behave differently over time. Some types advance more quickly than others. This can affect a person’s life expectancy and long-term prognosis. In general, people with pulmonary fibrosis due to autoimmune disease have a longer life expectancy compared to people with idiopathic pulmonary fibrosis. 

2. Age at diagnosis and overall health

The age of a person diagnosed with pulmonary fibrosis affects life expectancy. Younger people tend to live longer with pulmonary fibrosis. That’s because younger people have overall healthier and younger lungs. 

Also, younger people tend to have fewer health problems. Research shows that people with fewer health problems live longer with pulmonary fibrosis. So, older individuals who have fewer medical conditions often live longer than individuals of the same age who have other medical conditions. 

3. Pulmonary fibrosis stage 

Pulmonary fibrosis staging is based on how much damage has occurred in the lungs. The stages of pulmonary fibrosis are:

• Mild

• Moderate

• Severe

• Very severe

People with a lot of lung damage have a more advanced stage of pulmonary fibrosis. Life expectancy is shorter for people with severe pulmonary fibrosis compared to people with mild pulmonary fibrosis. 

4. Treatment 

There is no cure for pulmonary fibrosis, but treatment can help. 

Pulmonary fibrosis is a chronic, progressive disease, which means it causes a steady decline in lung function over time. But treatment can slow down the progression from mild to severe forms. This allows people to live longer. Medications like pirfenidone (Esbriet) and Ofev (nintedanib) can help slow down lung scarring. People who start treatment early and respond well to medications tend to live longer. 

Some people can also receive lung transplants. Lung transplants can help a person live longer. But lung transplant is a major surgery. People who have lung transplants need to take medication for the rest of their lives. 

Treatment for pulmonary fibrosis also includes preventing further lung damage. People with pulmonary fibrosis who smoke should quit. Getting vaccinated against lung infections like the flu, pneumococcal pneumonia, and COVID-19 can also help prevent lung damage and help people live longer.

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Written by:

Christine Giordano, MD

Christine Giordano, MD, is board-certified in general internal medicine. She received her medical degree from Rutgers New Jersey Medical School and completed residency at Thomas Jefferson University.

Edited by:

Patricia Pinto-Garcia, MD, MPH

Patricia Pinto-Garcia, MD, MPH, is a medical editor at GoodRx. She is a licensed, board-certified pediatrician with more than a decade of experience in academic medicine.

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