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IDIOPATHIC PULMONARY FIBROSIS

What is idiopathic pulmonary fibrosis (IPF)?

Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason. Over time, the scarring gets worse and it becomes hard to take in a deep breath and the lungs cannot take in enough oxygen.

IPF is a form of interstitial lung disease, primarily involving the interstitium (the tissue and space around the air sacs of the lungs), and not directly affecting the airways or blood vessels. There are many other kinds of interstitial lung disease that can also cause inflammation and/or fibrosis, and these are treated differently. It is important to work with your doctor to determine if you have IPF or another form of interstitial lung disease.

Causes

The cause of idiopathic pulmonary fibrosis is not completely understood. No one knows what causes idiopathic pulmonary fibrosis or why some people get it. “Idiopathic” means of unknown cause. Common risk factors for IPF include:

  • Genetics (family history): Up to 20% of people with IPF have another family member with an interstitial lung disease. If more than one member of your family has IPF, the disease is called familial pulmonary fibrosis.
  • Cigarette smoking: Approximately 75% of people with IPF are current or previous cigarette smokers.
  • Acid reflux (gastroesophageal reflux disease [GERD]): Approximately 75% of people with IPF have symptoms of acid reflux or heartburn.
  • Male sex: Approximately 75% of patients with IPF are male.
  • Age: Almost all patients with IPF are over the age of 50 years

Diagnosis

Although no single test can diagnose IPF, your doctor may recommend a number of different tests including:

  • Pulmonary function test (PFT): A PFT, or breathing test, is a simple test to measure how much air you can blow in and out of your lungs and how well your lungs can absorb oxygen.
  • Six-minute walk test: This test helps determine your physical fitness, as well as the amount of oxygen in your blood at rest and with physical activity.
  • Chest x-ray: Chest X-rays are simple images that can screen for interstitial lung disease and can sometimes be useful to help monitor progression.
  • Blood tests: A number of blood tests can be requested to help identify other causes of interstitial lung disease.
  • Computed tomography (CT scan): These radiology images will determine whether there is scarring in your lungs and will help identify the subtype of interstitial lung disease.
  • Bronchoscopy: Bronchoscopy is a procedure that involves inserting a small flexible tube, called bronchoscope, through your mouth or nose and into your lungs. This is not necessary for diagnosis but some patients with possible IPF undergo this procedure to help identify the presence of infection or to suggest other subtypes of interstitial lung disease.
  • Surgical lung biopsy: Patients with an unclear diagnosis will sometimes undergo a surgical lung biopsy performed by a surgeon under general anesthetic. This test involves small incisions in the side of the chest, which allows removal of lung tissue and its examination under a microscope.
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