Conditions with pulmonary fibrosis
There are many conditions that cause pulmonary (lung) fibrosis (scarring). However, only one of these is termed idiopathic pulmonary fibrosis.
The term “Interstitial lung diseases (ILDs)” refers to any condition that causes excess tissue affecting the lace-like network of supporting material (interstitium) around the air sacs of the lungs. The role of the lung is to deliver oxygen from those air sacs (alveoli) to the haemoglobin in blood vessels that carry it as fuel to the rest of the body. ILDs cause thickening of the tissues making it more difficult for the lungs to transfer oxygen to the blood vessels. The thickening can be permanent (fibrosis) or partially reversible, depending on the cause. Because of the greater difficulty transferring oxygen, affected individuals have to work harder to breathe or breath faster which contributes to a sensation of shortness of breath.1,2
There are more than 200 different ILDs and some component of fibrosis can have an important factor in many of these. Some ILDs can be caused by certain medications or drugs, occupational or environmental exposure, autoimmune conditions, or, many times, the cause is never discovered (idiopathic).1
An accurate diagnosis can take many years to establish in an individual with ILD and multiple tests can be required to rule out other conditions. Even when you have received a diagnosis, it’s important to remember that no two people will have the same experience with pulmonary fibrosis.1,3,4
Your doctor is best able to provide guidance and support tailored to your individual situation and suggest the best approach moving forward
British Lung Foundation. Pulmonary Fibrosis. Available at: https://www.blf.org.uk/support-for-you/pulmonary-fibrosis/. [Accessed February 2022].
Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res 2019;20(1).
Meltzer EB, et al. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008; 3:8.
Wells AU, et al. Interstitial lung disease guideline. Thorax 2008; 63: v1-v58.