Skip to main content

Explainer: Pulmonary Fibrosis

Pulmonary fibrosis is a group of more than 200 diseases marked by scarring in the lungs. Learn more during September, which is Pulmonary Fibrosis Month.

European Pulmonary Fibrosis Foundation Breathing Life poster

With every breath, flexible, healthy lungs expand to inhale essential oxygen. Air sacs in the lungs transfer oxygen into the bloodstream. But if someone has pulmonary fibrosis, scarring in the lungs can make breathing a struggle.

"Pulmonary" refers to the lungs, and "fibrosis" means scar tissue, which leads to decreased oxygen levels and stiffened lung tissue.

The normally thin, lacy walls of the air sacs in the lungs are no longer thin and lacy, but get thick, stiff and scarred,” according to the American Lung Association.

Patients experience breathlessness, especially during physical activity, as well as a persistent, dry cough; rapid, shallow breathing; unintentional weight loss, fatigue and other symptoms.

An estimated 3 million people worldwide are affected by idiopathic pulmonary fibrosis, the most common type of PF. Though progress has been made, current treatments only slow progression.

Check out Pulmonary Fibrosis Month campaigns from both the U.S. Pulmonary Fibrosis Foundation and the European Pulmonary Fibrosis Federation:

Embrace Your Breath

Breathing Life

PF Patients in Australia can find resources through Lung Foundation Australia and the Centre of Research Excellence in Pulmonary Fibrosis.

According to the U.S. PFF:

  • IPF affects 1 in 200 adults aged over 70 in the U.S.
  • More than 250,000 Americans are living with PFA.
  • Approximately 50,000 new cases are diagnosed every year.
  • Up to 40,000 Americans die from IPF each year.

Often there’s no known cause but autoimmune diseases and chemical exposures can cause the condition, according to the American Lung Association. Smoking increases the risk.

CSL is currently researching potential treatments for respiratory conditions and also helped launch the Open Source Imaging Consortium (OSIC). The research effort is using artificial intelligence to develop insights about how to diagnose and treat interstitial lung diseases, a category of lung disease that includes the 200+ types of pulmonary fibrosis.