Key takeaways:
Pulmonary fibrosis is a chronic lung condition caused by scarring in your lungs.
The average life expectancy for untreated pulmonary fibrosis is 3 to 5 years.
Life expectancy can depend on age, other medical conditions, the type of pulmonary fibrosis you have, and treatment.
Pulmonary fibrosis is a chronic lung condition that causes a 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’s 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 your lungs. Over time, fibrosis of the lungs makes it harder for them to expand and fill with air. It also becomes more difficult for oxygen to move from your lungs into your 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)
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 this number can change depending on a person’s specific situation.
It can be hard for healthcare professionals to give exact information about life expectancy. That’s because pulmonary fibrosis doesn’t always behave the same way over time. 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.
People with pulmonary fibrosis can also 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. This can include medications to treat cancer and autoimmune conditions.
Medical conditions: Certain autoimmune conditions, like lupus or 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.”
How types of pulmonary fibrosis affect prognosis
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 caused by an autoimmune disease have a longer life expectancy compared to those with idiopathic pulmonary fibrosis.
2. Age at diagnosis and overall health
Previous thinking was that younger people live longer with pulmonary fibrosis. But recent research suggests this may not always be true. One study found that people over age 70 and those younger than 60 have a similar survival rate relative to their age.
The more important factor may be overall health. Those with fewer health problems live longer with pulmonary fibrosis. So, older individuals who have fewer medical conditions often live longer than people of the same age who have other medical conditions.
3. Pulmonary fibrosis stage
Pulmonary fibrosis is staged based on how much lung damage has occurred. Healthcare professionals use in-office tests and imaging to determine severity. The stages of pulmonary fibrosis are:
Mild
Moderate
Severe
Very severe
People with a lot of lung damage have a more severe stage of pulmonary fibrosis. Life expectancy is shorter for people with severe pulmonary fibrosis compared to people with mild disease.
4. Treatment response
There’s no cure for pulmonary fibrosis, but treatment can help. People who start treatment early and respond well to medications tend to live longer. “Responding well” means the treatment is able to slow how quickly the condition progresses.
How treatment can affect prognosis
Pulmonary fibrosis is a chronic, progressive disease. This means it causes a steady decline in lung function over time. Treatment can slow down the progression from mild to severe forms, helping people live longer.
Medications
Medications can help slow down lung scarring. These medications can improve lung function, but they don’t cure the condition:
Pirfenidone (Esbriet)
Ofev (nintedanib)
Nerandomilast (Jascayd)
Research is ongoing for other medications. Involvement in a clinical trial might be an option for some people.
Pulmonary rehabilitation
Pulmonary rehabilitation is an outpatient program geared towards people with ongoing lung disease. It involves symptom management, counseling, breathing exercises, and other benefits. Studies suggest it can improve physical function and quality of life in people with pulmonary fibrosis.
Lung transplants
Some people can also receive a lung transplant. This can help a person live longer. But a lung transplant is a major surgery. People who have lung transplants need to take medication for the rest of their lives to prevent organ rejection.
Preventing further lung damage
Treatment for pulmonary fibrosis also includes preventing further lung damage. People with pulmonary fibrosis who smoke should quit.
Vaccinations can also help protect your lungs and improve life expectancy. Important vaccines include:
Flu
Pneumococcal pneumonia
COVID-19
Frequently asked questions
Currently, there’s no cure for pulmonary fibrosis. There are various treatments, programs, and support options that can slow the disease, help with your symptoms, and improve quality of life. And more treatments are in development.
Familial pulmonary fibrosis is hereditary. This is one type of pulmonary fibrosis that makes up about 20% of all cases. Researchers have identified some gene variants linked to this type, but not all. It’s likely that a combination of environment and genetics contributes to the development of pulmonary fibrosis.
Currently, there’s no cure for pulmonary fibrosis. There are various treatments, programs, and support options that can slow the disease, help with your symptoms, and improve quality of life. And more treatments are in development.
Familial pulmonary fibrosis is hereditary. This is one type of pulmonary fibrosis that makes up about 20% of all cases. Researchers have identified some gene variants linked to this type, but not all. It’s likely that a combination of environment and genetics contributes to the development of pulmonary fibrosis.
The bottom line
Pulmonary fibrosis is a progressive condition. This means it’ll worsen over time. But it can be difficult to predict how quickly that will happen. Life expectancy for people with pulmonary fibrosis depends on many factors, including the stage and type of pulmonary fibrosis. Other factors are what treatment they received and how well they respond to that treatment.
Why trust our experts?
References
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