The treatment of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and invariably fatal disease with a median survival of less than three years from diagnosis. The last decade has seen an exponential increase in clinical trial activity in IPF and this in turn has led to important developments in the treatment of this terrible disease. Previous therapeutic approaches based around regimens including corticosteroids and azathioprine have, when tested in randomized clinical trials, been shown to be harmful in IPF. By contrast, compounds with anti-fibrotic actions have been shown to be beneficial. Subsequently, the novel anti-fibrotic agent pirfenidone has, in many parts of the world, become the first treatment ever to be licensed for use in IPF. This exciting development, coupled with ongoing clinical trials of a range of other novel compounds, is bringing hope to patients and their clinicians and raises the prospect that, in the future, it may become possible to successfully arrest the development of progressive scarring in IPF.

Figure 1.. Low-power photomicrograph illustrating the characteristic appearances of usual interstitial pneumonia, the histological lesion of idiopathic pulmonary fibrosis (IPF)

The image shows fibroblastic foci overlying a region of micro-cystic honeycomb change. The sections have been stained with a Trichrome stain. This clearly highlights the extensive collagen (stained blue) and extracellular matrix deposition that occurs in IPF.

Figure 2.. A schematic outlining potential new idiopathic pulmonary fibrosis (IPF) therapies as they relate to the proposed pathogenetic events involved in the development of idiopathic pulmonary fibrosis

Modified from [29] with permission.