| Idiopathic Pulmonary Fibrosis - IPF |
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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.
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| What are the symptoms? |
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Shortness of breath, particularly with exertion |
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Chronic dry, hacking cough |
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Fatigue and weakness |
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Discomfort in the chest |
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Loss of appetite |
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Rapid weight loss
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| What is the prevalence of Pulmonary Fibrosis? |
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are at least 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. |
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| What are the causes? |
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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: |
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Inhaled environmental and occupational pollutants |
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Cigarette smoking |
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Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis |
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Certain medications |
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Therapeutic radiation |
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| How is it treated? |
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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.
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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. |
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Peter Scott - Team Leader
