Idiopathic pulmonary fibrosis is a relatively rare condition that appears to have increased in prevalence in recent years. It has become an exciting area to work in because of the potential for good clinical management and the growth in therapeutic options.
What is IPF?
IPF is one of the interstitial lung diseases, a group of conditions that includes sarcoidosis, the chronic pneumonias and other fibrotic processes.
As the name suggests, IPF is not an airways disease like COPD and asthma, but a disease of the interstitium, which is the matrix of the lung. This difference needs to be carefully explained to people diagnosed with IPF because they often consider chest problems to be similar to asthma and COPD and find it hard to understand why inhalers will not be beneficial, unless they also have asthma or COPD.
It is idiopathic disease –there appears to be no known cause for the development of the fibrosis.1 Currently IPF is a progressive disease without a cure.
IPF is not always picked up on a chest X-ray so a normal X-ray does not exclude the diagnosis. IPF tends to have a specific radiological pattern, including reticular opacities, traction bronchiectasis and honeycombing usually at the bases of the lungs seen on high resolution CT imaging (HRCT).