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
-
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 contact us at:
Pulmonary Fibrosis
Foundation
1332 North Halsted Street Suite 201
Chicago, Illinois 60642-2642
(312) 587-9272 fax (312) 587- 9273
Last
updated
June 27, 2008 |