What is Pulmonary Fibrosis?

Pulmonary Fibrosis is a disease of inflammation that results in scarring, or fibrosis, of the lungs. In time, pulmonary fibrosis can build up to the point where the lungs are unable to provide oxygen to the tissues of the body.

Doctors use the word "idiopathic" (from the Greek "idio" meaning "peculiar" or "unusual" and "pathy" meaning "illness") to describe the disease, because the cause of IPF is usually unknown. Currently, researchers believe that idiopathic pulmonary fibrosis may result from either an autoimmune disorder, a condition in which the body's immune system attacks its own tissues, or the after effects of an infection, most likely a virus.

Whatever the trigger is for idiopathic pulmonary fibrosis, it appears to set off a series of events in which the inflammation and immune activity in the lungs--and, eventually, the pulmonary fibrosis processes, too--become uncontrollable. In a few cases, heredity appears to play a part, possibly making some individuals more likely than others to get idiopathic pulmonary fibrosis. While the exact cause remains unknown, associations have been made with the following:

• Inhaled environmental and occupational pollutants
• Asbestos
• Scleroderma
• Rheumatoid Arthritis
• Lupus
• Sarcoidosis
• Certain medications
• Therapeutic radiation

In studies of patients with idiopathic pulmonary fibrosis, the average survival rate has been found to be 4 to 6 years after diagnosis. Those who develop idiopathic pulmonary fibrosis at a young age seem to have a longer survival.

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