Banner: About IPF

What is IPF?

IPF stands for Idiopathic Pulmonary Fibrosis, a rare condition that affects 14 - 43 people per 100,000, with a total of approximately 3 million patients worldwide.1,2 The lung tissue of someone with IPF becomes thickened, stiff and scarred (Figure 1). Fibrosis is the medical term for this scarring.3 The tiny air sacs (known as alveoli) and blood vessels of the lungs are responsible for providing the oxygen that every part of the body needs.

Patient guide

Backgrounder: About IPF


Backgrounder: About IPF

Figure 1. The normal lungs and what happens to the lungs in IPF


IPF becomes worse over time and as the lung tissue becomes more and more scarred it is increasingly difficult for oxygen to get into the bloodstream through the lungs. This results in a shortness of breath and the organs not getting enough oxygen to function normally.1,4

Sometimes doctors can find out what is causing lung scarring. For example, exposure to environmental pollutants and certain medicines can cause the disease.2 However, in most cases of lung scarring, an exact cause is never established – and that is then what we call Idiopathic Pulmonary Fibrosis.2

Certain factors, such as cigarette smoking, some viral infections, genetics and GERD (gastro-oesophageal reflux disease) may increase the risk of IPF.5

The signs and symptoms of IPF include:2

  • Shortness of breath - only during exercise at first.
  • Dry, hacking cough that doesn't get better.
  • Rapid, shallow breathing.
  • Gradual, unintended weight loss.
  • Tiredness and generally feeling unwell.
  • Aching muscles and joints.
  • Clubbing (widening and rounding) of the tips of the fingers or toes.
  • "Velcro-like" crackles heard by the physician during lung auscultation.

What is IPF?   

Expert video:  What is IPF?

What happens to the lungs in IPF?

Expert video: What happens to the lungs in IPF?

What are rare diseases and what do we know about them?      

What are rare diseases and what do we know about them?



  1. Raghu G, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006;174:810–816.
  2. Meltzer EB, et al. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008;3:8.
  3. Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824.
  4. Woodcock HV, et al. The treatment of idiopathic pulmonary fibrosis. F1000prime Rep 2014;6:16.
  5. Fernandez IE, et al. New cellular and molecular mechanisms of lung injury and fibrosis idiopathic pulmonary fibrosis. Lancet 2012;380:680–688.