Some Basic Facts
Idiopathic pulmonary fibrosis (IPF) is a debilitating disease marked by progressive scarring of the lungs that gradually interferes with a person's ability to breathe and thus obtain enough oxygen for vital organs to function normally. IPF is one of approximately 200 diseases—collectively called interstitial lung diseases (ILDs)—in which lung scarring is found. The lung scarring that occurs in ILDs is referred to as pulmonary fibrosis.
Sometimes pulmonary fibrosis can be linked to a particular cause, such as:
- Exposure to inhaled inorganic dusts (e.g., asbestos fibers) or organic dusts (e.g., molds, fungi)
- Chemotherapy or radiation therapy
- Lung infection
- Connective tissue disease, such as scleroderma or rheumatoid arthritis
In the majority of pulmonary fibrosis cases, however, no known cause can be established. Until recently, these cases were typically grouped together and called idiopathic pulmonary fibrosis ("idiopathic" means "of unknown cause"). Consequently, your doctor may have referred to your condition by one of a number of names that are sometimes used to describe IPF. This confusion also may be due, in part, to similarities between IPF and the other ILDs. However, with new guidelines developed by physicians for classification and diagnosis, IPF is now considered a distinct clinical disorder with its own specific characteristics and outcomes.
Approximately 128,000 people in the United States have IPF, and an estimated 48,000 new cases develop each year. The disease tends to affect men more than women and usually strikes people between the ages of 50 and 70.
IPF hinders a person's ability to absorb oxygen. This makes them feel short of breath. The injury to the lungs may also cause a dry cough. As the scarring gets worse, patients become out of breath with lower levels of effort (e.g., while dressing and undressing). Unfortunately, IPF is ultimately disabling and fatal.
IPF May Also Be Referred to as:
- Cryptogenic fibrosing alveolitis
- Diffuse fibrosing alveolitis
- Interstitial diffuse pulmonary fibrosis
- Usual interstitial pneumonia
What Causes IPF?
While the cause of IPF remains a mystery, we do know that IPF involves an exaggerated or uncontrolled healing response that, over time, produces fibrous scar tissue. This scarring surrounds the normally thin-walled small air sacs of the lung (called alveoli), making them thicken and become stiff. This prevents oxygen from getting from the air sacs into nearby blood vessels that deliver the oxygen to your body.
Exactly what starts the cycle of injury and abnormal healing is unknown. One possibility is that the body's normal immune response, which reacts to foreign substances, becomes overactive or malfunctions.
Researchers are investigating a number of potential risk factors that may make a person more likely to develop IPF. According to a recent consensus statement by several international medical organizations, these risks may include cigarette smoking, exposure to antidepressants, gastroesophageal reflux, occupational exposure to dusty environments, viral infection, and inherited predisposition (a family history of pulmonary fibrosis). Some of these potential risk factors have only recently been identified and more research is needed to determine if they truly are factors in the development of IPF.
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