New treatment and markers of prognosis for idiopathic pulmonary fibrosis: lessons learned from translational research

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease with increasing prevalence, high mortality rates and poor treatment options. The diagnostic process is complex and often requires an interdisciplinary approach between different specialists. Information gained over the past 10 years of intense research resulted in improved diagnostic algorithms, a better understanding of the underlying pathogenesis and the development of new therapeutic options. Specifically, the change from the traditional concept that viewed IPF as a chronic inflammatory disorder to the current belief that is primarily resulting from aberrant wound healing enabled the identification of novel treatment targets. This increased the clinical trial activity dramatically and resulted in the approval of the first IPF-specific therapy in many countries. Still, the natural history and intrinsic behavior of IPF are very difficult to predict. There is an urgent need for new therapies and also for development and validation of prognostic markers that predict disease progression, survival and also response to antifibrotic drugs. This review provides an up to date summary of the most relevant clinical trials, novel therapeutic drug targets and outlines a spectrum of potential prognostic biomarkers for IPF.