What is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis (IPF) is a progressive disease (gets worse over time) isolated to the lung. It is a type of interstitial lung disease, which is a group of 200 diseases with similar symptoms but different causes. Currently, more than 80,000 adults in the United States have IPF, and more than 30,000 new cases are diagnosed each year.

In IPF, lung tissue becomes scarred and changes the lung's ability to function normally. The scarring typically starts at the edges of the lungs and advances towards the center of the lungs. Typically, mild scarring occurs first, but over months to years, the normal lung tissue is replaced by more heavily scarred lung tissue, which makes it difficult to breathe and deliver needed oxygen to the body. Unfortunately, IPF is a disabling disease without a known cure and with few treatment options.

What causes idiopathic pulmonary fibrosis?

The cause of IPF is unknown, but the following are possible risk factors for the disease:

  • Working around dust or fumes. Farmers, ranchers, hairdressers, stone cutters/polishers, and metal workers face a moderately increased risk of developing the disease because of exposure to these dusts or occupational fumes.
  • Age between 40 and 70 (approximately 2/3 of patients are older than 60 at the time of diagnosis).
  • History of smoking.
  • Male gender (more men are diagnosed with the disease than women).
  • Genetics (inheriting the disease from parents).

What are the symptoms of idiopathic pulmonary fibrosis?

Symptoms of IPF usually develop gradually and may not be noticed until the disease is well-established. IPF affects each person differently and the disease progresses at varying rates. Symptoms include:

  • Dry cough.
  • Shortness of breath, especially during or after physical activity.
  • Fatigue.
  • Weight loss.
  • Clubbing, a widening and rounding of the tips of the fingers.
  • Swelling of the legs.

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