Emerging drugs for idiopathic pulmonary fibrosis

Abstract

Pulmonary fibrosis is often the end stage of chronic, persistent, low-level lung injury, either of known or unknown cause. The most severe form of pulmonary fibrosis is idiopathic pulmonary fibrosis (IPF), a disease process of unknown aetiology and one that often leads to respiratory failure and death. At present there are no proven or effective drug therapies for IPF. Recent advances in understanding of disease pathogenesis have focused attention on drug targeting of fibrogenic pathways, as opposed to traditional anti-inflammatory approaches. In this report, the present status of drug development of a number of emerging antifibrotic strategies and agents that may prove more effective in the therapy of this progressive, debilitating and fatal disease are reviewed.

Keywords::

• bosentan
• connective tissue growth factor
• corticosteroids
• eicosanoids
• endothelin-1
• etanercept
• idiopathic pulmonary fibrosis
• IFN-γ
• inflammation
• myofibroblasts
• N-acetyl cysteine
• oxidative stress
• pirfenidone
• protein kinase inhibitors
• repair
• tetrathiomolybdate
• TGF-β
• TNF-α
• usual interstitial pneumonia
• zileuton