Progressive pulmonary fibrosis (PPF) occurs when lung scarring (fibrosis) worsens over time in some people living with interstitial lung disease (ILD). As scarring increases, the lungs become less able to function properly, which can lead to worsening symptoms and declining lung function.
PPF is not a specific diagnosis. Instead, it describes a pattern of worsening lung scarring that can occur in several types of interstitial lung disease (ILD). It most often develops in people with fibrotic interstitial lung diseases and is often referred to as non-IPF ILDs. The rate of progression can vary from person to person, making regular monitoring by your healthcare provider an important part of care.
Key Facts About PPF
- PPF is sometimes referred to as progressive fibrotic interstitial lung disease (PF-ILD) or a progressive fibrosing phenotype.
- The lung scarring caused by PPF is irreversible, meaning the scarring is permanent once it happens.
- PPF can continue to worsen over time, even when the underlying ILD is being treated.
- Not everyone with ILD develops PPF.
What are the Symptoms of PPF?
Early in the disease, you may have no symptoms or mild symptoms such as shortness of breath and a cough. As the condition progresses other signs and symptoms may include:
- Increased shortness of breath during activities of daily living and exercise
- A dry cough that does not get better or worsens
- Feeling unusually tired
- Clubbing, which causes the tips of the fingers or toes to become wider and rounder than normal
- Losing weight without trying
PPF is important to identify early because it can lead to worsening symptoms, loss of lung function and reduced quality of life. Early diagnosis and treatment may help slow disease progression.
How is PPF Diagnosed?
Your healthcare provider may diagnose progressive pulmonary fibrosis if your lung scarring and symptoms have worsened over the past year. To determine if you have PPF, at least two of the following three signs of progression must be present:
- Worsening symptoms, such as increased shortness of breath and cough.
- A decline in how well the lungs work, shown by lung function tests.
- An increase in lung scarring seen on imaging tests such as a high-resolution CT scan.
How is PPF Treated and Managed?
While there is currently no cure for progressive pulmonary fibrosis, treatment can help slow the progression of the disease, manage symptoms, address the underlying lung disease contributing to the scarring and improve quality of life. Because PPF can develop in several different types of ILD, treatment often begins with managing the underlying condition.
If you have PPF, it is important to work with a lung specialist, called a pulmonologist, who has experience treating interstitial lung diseases (ILDs). An ILD specialist can help confirm the diagnosis, monitor disease progression and recommend the most appropriate treatment options.
Your treatment plan will be individualized based on the severity of the disease, symptoms you are experiencing and other health conditions you may have, also called comorbidities. Management of PPF requires a team approach, and the goal is to slow the rate of lung scarring, reduce inflammation and manage unwanted symptoms. Treatment may include the following:
Who is at Risk for PPF?
Some people with ILD are more likely to develop PPF. Factors that may increase the risk include:
- being male
- having a history of or currently smoking
- being older
- having lower lung function on breathing tests
- having more extensive lung scarring on CT scans
- having a family history or genetic factors that increase the risk of lung scarring
Get support from Lung Health Navigators
Living with PPF can feel overwhelming—you don’t have to manage it alone. Connect with trained Lung Health Navigators for one-on-one support, answers and guidance.
Page last updated: August 21, 2026
