Deregulation of microRNA Processing in Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease with an incidence in the United States of 42.7 to 63 per 100,000 and an incidence in Europe of 16.3 to 17.4 per 100,000 (Nalysnyk et al. 2012). Incidence and severity of IPF increases with age, but on average projected survival is just 3-4 years post-diagnosis (Raghu et al. 2014). Until recently, treatment options have been limited and mostly palliative, with little impact on survival. For many years, the only established treatment shown to extend life was lung transplantation. However, lungs are the most difficult organs to transplant, with patients surviving an average of just 4.6 years post-transplantation (NIH 2014). Just recently, two drugs (pirfenidone and nintedanib) have been approved for treatment of IPF. However, even with such treatments the unyielding progression of IPF is only slowed, and a more effective treatment for IPF remains both necessary and illusive (as reviewed in Jenkins and Goodwin 2014).