|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 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis
the actual numbers may be significantly higher. Of these more than 40,000 expire annually.
This is the same as die from Breast Cancer. 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
• 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. Contact us at:
Pulmonary Fibrosis Foundation
1332 North Halsted Street Suite 201
Chicago, Illinois 60622
(312) 587-9272 fax (312) 587- 9273 for more information.
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