Figure 1- Idiopathic Pulmonary Fibrosis definition (adapted from- ¹)

Figure 1- Idiopathic Pulmonary Fibrosis definition (adapted from- ¹)

Idiopathic Pulmonary Fibrosis (IPF) is a rare, long-term, progressive disease that affects the fragile tissue in the lungs. It leads to a gradual, persistent decline in lung function.² IPF typically occurs in people who are more than 45 years of age, and the average patient age is 65.³ The disease is more common in men than women.⁴ About 110,000 people in Europe have IPF, and 35,000 new patients are diagnosed each year.⁵ The disease is irreversible, and without treatment, half of IPF patients will die within 2-5 years of diagnosis.⁶

Figure 2: Understanding what Idiopathic Pulmonary Fibrosis is (adapted from: ⁷

Figure 2: Understanding what Idiopathic Pulmonary Fibrosis is (adapted from: ⁷

How does IPF happen?

In IPF, damage to the cells that line the air sacs, or ‘alveoli’, of the lungs leads to the formation of scar tissue. This is why IPF is one of the interstitial lung diseases (ILDs). Interstitial lung disease describes a large group of disorders characterised by progressive scarring of the lung tissue between and supporting the air sacs. In IPF, scar tissue builds up around the alveoli and this makes it harder for oxygen to pass into the bloodstream.⁸ As a result, the brain, heart and other vital organs may not get the amount of oxygen they need to work properly.⁹ The amount of scarring usually increases over time, although how quickly it progresses varies. The scarring is generally irreversible.

 

 
Figure 3: Location of lungs and airways in the body (adapted from: ¹⁰)

Figure 3: Location of lungs and airways in the body (adapted from: ¹⁰)

 

Figure 4: How Idiopathic Pulmonary Fibrosis affects the lungs tissue (adapted from: ¹¹)

Figure 4: How Idiopathic Pulmonary Fibrosis affects the lungs tissue (adapted from: ¹¹)

Figure 5: Difference between the tissue of healthy lungs and affected by IPF lungs; and examples of X-rays taken of healthy lungs and affected by IPF lungs (adapted from: ¹² ¹³)

Figure 5: Difference between the tissue of healthy lungs and affected by IPF lungs; and examples of X-rays taken of healthy lungs and affected by IPF lungs (adapted from: ¹² ¹³)