Pulmonary Fibrosis Foundation

What is Pulmonary Fibrosis?

Pulmonary Fibrosis involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissue’s ability to transfer oxygen into the bloodstream.

What are the symptoms?

Shortness of breath, particularly with exertion
Chronic dry, hacking cough
Fatigue and weakness
Discomfort in the chest
Loss of appetite
Rapid weight loss

What is the prevalence of Pulmonary Fibrosis?

There are five million people worldwide that are affected by this disease. In the United States there are over 500,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group.

What are the causes?

Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following:

• 
  Inhaled environmental and occupational pollutants
  Cigarette smoking
  Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis
  Certain medications
  Therapeutic radiation

How is it treated?

There are currently no effective treatments or a cure for Pulmonary Fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress.

Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly.

There are a number of new trials testing drugs to treat Pulmonary Fibrosis. For more information visit http://www.clinicaltrials.gov or you can contact us at:
Pulmonary Fibrosis Foundation1332 North Halsted Street Suite 201Chicago, Illinois 60642-2642(312) 587-9272 fax (312) 587- 9273

Last updated June 30, 2009

More details on the foundation visit:

http://pulmonaryfibrosis.org/ipf.htm