Cause of pulmonary fibrosis

The type of fibrosis you are diagnosed with will often indicate the cause. For example, if you are diagnosed with hypersensitivity pneumonitis your lungs and immune system are reacting to an inhaled cause such as mould, bird feather proteins or dusts. If your PF is related to an overactive immune system, such as in Lupus and RA, then these diseases are the cause. When all causes have been investigated and nothing has been identified patients are classed as idiopathic (unknown cause).

Doctors and researchers are still investigating all the biological pathways and processes involved in the production of fibrosis (scarring) in the lungs. A lot has been discovered and research is continuing. This can lead to anxiety when you are first diagnosed, so always discuss any questions regarding your health with your consultant or nurse to try to alleviate any worries.

Idiopathic pulmonary fibrosis

There are many theories about what triggers IPF, but no firm conclusions.

It usually affects people over the age of 50 years and is slightly more common in men than women. It occurs globally and is not related to social or economic status. Acid reflux, infections and smoking, as well as employment, family and environmental factors, have all been linked to IPF:

  • IPF patients often suffer from acid reflux (heartburn). However, heartburn is much more common than IPF and most people who experience heartburn will never go on to develop IPF
  • A lot of patients report a nasty chest infection leading to a lasting cough – often prompting them to visit their GP
  • A direct link to smoking has not been proven, however a large percentage of IPF patients have smoked at some point in their lives and many people over 50 will have been exposed to second-hand smoke
  • Inhaling dust minerals at work such as coal and saw dusts has been linked to IPF
  • Research into family history is ongoing. The link is still unclear but there are families where siblings have developed IPF
  • Dusty polluted environments are commonly associated with IPF
  • Ageing-IPF is a disease usually of older people. Research into ageing and IPF is developing.

Action for Pulmonary Fibrosis has committed nearly £600,000 to research in the last year, all of which was donated by our amazing supporters and fundraisers. This research includes two major projects, one looking at genetic insights, and another exploring the role played by bacteria in the lungs. Read more about our research.