What is pulmonary fibrosis?
Pulmonary fibrosis is the end result of many different conditions that cause scar tissue to build up in your lungs and make breathing increasingly difficult. All types of pulmonary fibrosis are considered rare.
Pulmonary fibrosis is an interstitial lung disease (ILD). There are more than 200 different ILDs.
- pulmonary: it affects your lungs
- fibrosis: a build-up of scar tissue, which makes your lungs stiff
- interstitial: affects your interstitium, the network of tissue that supports the air sacs in your lungs
What are interstitial lung diseases?
Interstitial lung diseases cause scarring in your lungs, inflammation in your lungs or a mix of both. Some mostly cause scarring. Some mostly cause inflammation. Often there is a combination of scarring and inflammation. It’s important to know which is the major cause of your symptoms to help decide the best treatment for you.
Causes and related conditions
Some types of pulmonary fibrosis have an identifiable cause. But for many types, a definite cause cannot be found, for example idiopathic pulmonary fibrosis.
Although we do not always know what causes pulmonary fibrosis, we do know it is not a form of cancer or cystic fibrosis, and it is not contagious. Cystic fibrosis is also not a type of ILD or pulmonary fibrosis.
This information covers the conditions seen most often:
- idiopathic pulmonary fibrosis or IPF
- hypersensitivity pneumonitis formerly called extrinsic allergic alveolitis
- pneumoconiosis, also known as an occupational interstitial lung disease
- pulmonary fibrosis associated with connective tissue and autoimmune diseases
- drug-induced pulmonary fibrosis
Questions about diagnosis and treatment?
The treatment and outlook for different types of pulmonary fibrosis vary considerably, so if you’re not sure about your diagnosis, check with your doctor or nurse for the exact name of your lung condition.
Not sure what to ask?
If you are unsure what to ask your health care professional when you have, or could have, pulmonary fibrosis, try reading this helpful list. It suggests questions to ask for diagnosis, treatments and much more.