What is idiopathic pulmonary fibrosis?

By June 17, 2019Chest & Lung

Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason. Over time, this scarring gets worse making it hard to and it becomes hard breathe effectively.

IPF affects approximately three million people worldwide. The disease mostly affects patients over the age of 50 and more men than women.

Risk factors may include:

  • Smoking.
  • Lung injury.
  • History of smoking.
  • Genetics.
  • Abnormal acid reflux.
  • Environmental exposure like working around dust or fumes.
  • Chronic viral infections.

Symptoms of IPF usually develop gradually and may not be noticed until the disease is well-established.

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.

Diagnosis


Idiopathic pulmonary fibrosis (IPF) can be difficult to diagnose. Many lung diseases can cause breathlessness and cough, and some of these diseases have similar symptoms and X-ray results. You may have to see more than one specialist, including a respirologist, to help with the diagnosis.

Your doctor will ask you questions about your home and work environment, as well as your smoking and medical history.

Although no single test can diagnose IPF, your doctor may recommend a few different tests. A physical exam of your lungs will be done to assess other parts of your body including your heart, joints, fingernails, skin and muscles.

Tests include:

  • Pulmonary function test (PFT): A PFT, or breathing test, measures 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 helps determine your physical fitness, and the amount of oxygen in your blood at rest and with physical activity.
  • Chest X-ray: Chest X-rays are images that can screen for interstitial lung disease and can sometimes be useful to help monitor progression.
  • Blood tests: Your doctor may request several blood tests to help identify other causes of interstitial lung disease.
  • Computed tomography (CT scan): The radiology images will determine if there’s scarring in your lungs and help identify the subtype of interstitial lung disease.
  • Bronchoscopy: Bronchoscopy is a procedure where a small flexible tube called a bronchoscope is inserted through your mouth or nose and into your lungs. Some patients with possible IPF undergo this procedure to help identify the presence of infection or to look out for other subtypes of interstitial lung disease.
  • Surgical lung biopsy: When a patient has an unclear diagnosis, they may undergo a surgical lung biopsy. This test involves small incisions in the side of the chest, and the removal of lung tissue for an examination under a microscope.
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Treatment 


Work with your family doctor or pulmonologist (lung specialist) to find the best treatment for you. There’s no cure for IPF and there are currently no procedures or medications to remove the scarring from the lungs. Treatments are used to slow the progression of the lung and manage the other symptoms of the disease.

There are currently two medications approved for use in IPF.

Pirfenidone, an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis that slows the progression of IPF. Some patients who take Pirfenidone have side-effects, most commonly stomach upset and skin rashes, particularly with exposure to the sun.

Nintedanib, an anti-scarring (anti-fibrotic) medication that slows the progression of IPF. Some patients taking Nintedanib have side-effects, most commonly including diarrhoea.

Lifestyle changes


IPF is an irreversible and progressive disease. It will be crucial to learn good coping skills and educate yourself about the disease as much as possible.

  • Quit smoking. It will help slow down the progression of any lung disease.
  • Get a flu shot each year.
  • Ask your doctor about getting a pneumonia shot.
  • Keep away from people with chest infections and colds.
  • Wash your hands properly and regularly.
  • Stay active and fit.

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