What Is Pulmonary Fibrosis?

According to the National Institutes for Health (NIH), PF affects 200,000 Americans(1)(2). PF is a condition in which the lung tissue becomes thickened, stiff, and scarred1. The development of the scar tissue is called fibrosis. As lung tissue becomes scarred and thicker, it loses its ability to transfer oxygen into the bloodstream. As a result, the brain and other organs don’t get the oxygen they need. In some cases, doctors can determine the cause of the fibrosis, but in most cases, there is no known cause. When there is no known cause for the fibrosis the disease is called idiopathic pulmonary fibrosis or IPF.

To find out more about pulmonary fibrosis, please visit our Resources page.

Fast Facts

  • This disease affects 200,000 people in the US(1)(2). Alarmingly, prevalence has increased as much as 150% since 2001(3)(4).
  • The median survival rate is just two to three years, and more than 2/3 of patients will die within five years (5).
  • There is no known cause, no FDA-approved treatment and no cure for PF.
  • An estimated 40,000 people die each year from PF – one every 13 minutes (approximately the same number that die annually from breast cancer).
  • An estimated 48,000 new cases are diagnosed each year(1).
  • PF can strike anyone, but the disease tends to affect men more than women and usually occurs between the ages of 50 and 70(2).
  • PF is four times more common than cystic fibrosis, yet it remains virtually unknown to the public, many primary  care physicians and policymakers.
  • A study by the American Lung Association (ALA) indicates that mortality rate from PF has increased significantly, especially in women, and is predicted to continue to rise(6).


  1. National Institutes of Health web page (www.nhlbi.nih.gov), 2010
  2. Coultas DB, Zumault RE, Black W, Sobonya RE. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. 1994; 150:967-72.
  3. Ganesh Raghu, M.D., Derek Weycker, Ph.D., John Edelsberg, M.D., M.P.H., Williamson Z. Bradford, M.D., Ph.D., Gerry Oster, Ph.D.; Incidence and Prevalence of IdiopathicPulmonary Fibrosis; AJRCCM: doi:10.1164/rccm.200602-163OC. Published June 29, 2006: (Utilization data revised to NIH estimated prevalence of 128,000/200,000 prevalance &annual cost of 33734 per patient using broad definition.)
  4. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment (international consensus statement). Am J Respir Crit Care Med. 2000;161:646-664.
  5. Brett Ley, MD, Harold Collard, MD, Talmadge E. King, MD; Clinical Course and Prediction of Survival in Idiopathic Pulmonary Fibrosis; AJRCCM Articles in Press. Published on October 8, 2010 as doi:10.1164/rccm.201006-0894CI
  6. American Lung Association Lung Disease Report, c. 2008