| Idiopathic Pulmonary Fibrosis |
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SYMPTOMS
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 number as that of people who 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 also indicates that many infants are afflicted by Pediatric Interstitial Lung Disease . At this time there is limited data on prevalence for this group. 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:
TREATMENTS 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 longevity of patients after diagnosis varies greatly. |
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