Pulmonary fibrosis represents the end stage of a number of heterogeneous conditions and is, to a greater or lesser degree, the hallmark of the interstitial lung diseases (ILDs). It is characterised by the excessive deposition of extracellular matrix proteins within the pulmonary interstitium leading to the obliteration of functional alveolar units and in many cases, respiratory failure. While a small number of ILDs have known aetiologies, most are idiopathic in nature, and of these, idiopathic pulmonary fibrosis (IPF) is the most common and carries with it an appalling prognosis – median survival from the time of diagnosis is less than 3 years.
This reflects the lack of any effective therapy to modify the course of the disease, which in turn is indicative of our incomplete unders...

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