Review Series – Inflammation & Fibrosis Treatments in idiopathic pulmonary fibrosis: time for a more

Idiopathic pulmonary fibrosis (IPF) is a progressive age-related lung disease, the cause of which is not been fully understood. IPF is a devastating disease with mortality worse than many cancers, and treat-ment options are limited. IPF is thought to occur after recurrent injury to the alveolar epithelium fol-lowed by abnormal repair characterized by the for-mation of fibroblast and myofibroblast foci and excessive deposition of extracellular matrix. An updated classification of the idiopathic interstitial pneumonias has encouraged a large number of clinical trials. On the whole, these have disap-pointed. Improvements in molecular techniques have developed our understanding of IPF and with it identified new pathways and potential targets for therapeutic intervention. These insights are lead-ing to interest in biomarkers of disease progres-sion and prognosis and to novel anti-fibrotic agents and a more targeted approach to the treat-ment of IPF