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. 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?

Some theories have speculated 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

Because the origin and development of the disease is not completely understood, misdiagnosis is common. 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.