Emerging therapeutic interventions for idiopathic pulmonary fibrosis.Expert Opin Investig Drugs 2014; 23(7):893-910EO
Idiopathic pulmonary fibrosis (IPF) is a devastating and relentlessly progressive lung disorder. Previously, it was thought to be a chronic inflammatory disease; however, it is now considered to be an epithelial-fibroblastic disease. In accordance with this paradigm change, efforts toward the development of novel therapeutic targets for IPF have acquired a new direction. Currently available therapies are largely ineffective in reversing the lung damage, and lung transplantation is the only effective treatment for end-stage disease. Limitations in advancement of IPF therapeutics are due to a poor understanding of its pathogenesis, unavailability of reliable animal models and slow disease progression. Recent research on IPF has resulted in the identification of a plethora of novel targets that are in various stages of development and offers hope that in the near future that there will be better therapeutic options available for the treatment of IPF.
This review discusses existing therapies and highlights some of the recent, novel therapeutics being explored in the current clinical landscape for the treatment of this chronic, disabling disorder. The review also discusses the pathogenic rationale behind current therapies.
Targeting one fibrotic signaling pathway at a time may not have any significant effect on the control of IPF. It is therefore recommended that future IPF management focuses on targeting multiple pro-fibrotic pathways associated with its complex pathogenesis.