Idiopathic Pulmonary Fibrosis

It is useful to understand what the following words mean:

  • Idiopathic means ‘of unknown cause’.
  • Pulmonary means ‘affecting the lungs’.
  • Fibrosis means ‘thickening’ or ‘scarring’.

IPF is a buildup of scar tissue in the lungs. The scar tissue damages the lungs and makes it hard for oxygen to get in. Not getting enough oxygen to the body can cause serious health problem and even death.

Causes of IPF are unknown. However, smokers and patients with a family history of IPF are more prone to get the disease.

IPF is generally seen in individuals above 60 years of age and is more common in men than women. However, it can affect anybody in any age.

Symptoms tend to develop gradually:

  • Shortness of breath that gradually becomes worse is the main symptom. This is due to the reduced amount of oxygen that gets into the bloodstream. Shortness of breath on exertion may be noticed at first.
  • A dry cough often develops (a cough with little or no sputum).
  • Finger or toenail clubbing occurs in about half of cases. Clubbing is a painless swelling at the base of the nails.
  • An increased risk of developing chest infections.

As the condition progresses, symptoms can sometimes quickly become worse (called an acute exacerbation).

The above symptoms, combined with crackles heard by a doctor listening to your lungs with a stethoscope, can suggest the diagnosis. Other specialized tests include a high resolution CT scan of the chest (HRCT), pulmonary function tests and rarely a lung biopsy.

No drugs exist to reverse the scarring process that has already occurred in the lung. A new drug called pirfenidone has shown to slow the process of lung scarring. Another antifibrotic drug called Nintedanib is currently under research. Other treatment options include supplemental oxygen, pulmonary rehabilitation, smoking cessation, vaccination and lung transplantation at advanced centres.