Pulmonary fibrosis (PF) describes a condition in which the lung tissue becomes thickened, stiff, and scarred.The medical term used to describe this scar tissue is fibrosis. The air sacks (alveoli) and blood vessels within the lung are responsible for delivering oxygen to the body, including the brain , heart, and other organs. As lung tissue becomes scarred and thickened, it is difficult for the lungs to transfer oxygen into the bloodstream. As a result, the brain, heart, and other organs don’t get the oxygen they need to function properly . In some cases, doctors can determine the cause of the PF, but in most cases, the cause remains unknown . When there is no known etiology (cause) for the development of PF (and certain radiographic and/or pathologic criteria are met) the disease is called idiopathic pulmonary fibrosis or IPF.

A Snapshot of the Disease

  • In the United States (U.S.), IPF affects between 132,000-200,000 people.
  • In the U.S., an estimated 50,000 new cases are diagnosed each year.
  • In the European Union, IPF affects between 37,000 and 40,000 people.
  • In the United Kingdom alone , more than 5,000 new cases of IPF are diagnosed each year.
  • PF can strike anyone, but the diseases tends to affect men more than women and usually occurs between the ages of 50 and 70.

The development of pulmonary fibrosis is also associated with other conditions :

  • Autoimmune diseases such as scleroderma, lupus, and rheumatoid arthritis.
  • Occupational and environmental exposures such as asbestos, silica, animal proteins, gasses , and fumes.
  • Certain medications and therapeutic treatments; a few examples are cancer medications (bleomycin,cytoxan), radiation theraphy, cardiac medications (propanolol, amiodarone), and sulfa containing antibiotics for urinary tract infections.
  • Pulmonary infections (or recurrent pulmonary infections) both viral and bacterial.
  • Gastroesophageal reflux disease (GERD).
  • Genetic factors and cigarette smoking (can increase an individual’s risk of developing PF).

What is the Mortality Rate of Pulmonary Fibrosis?

  • An estimated 40,000 people die in the U.S. each year from PF. The median survival rate is just two to three years following diagnosis, and more than two-thirds of patients will die within five years.
  • The mortality rate from PF has increased significantly, especially in women, and is predicted to continue to rise.

For more information about pulmonary fibrosis and the Pulmonary Fibrosis Foundation,
please visit www.pulmonaryfibrosis.org or call 888.733.6741 or +1 312.587.9272