Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive diffuse 
parenchymal lung disease of unknown origin, with a mortality rate exceeding that 
of many cancers. The diagnostic process is complex and relies on the clinician 
integrating clinical, laboratory, radiological, and histological data. In the 
last decade, major advances in our understanding of the pathogenesis of IPF have 
shifted the paradigm from a primarily inflammatory process evolving to fibrosis 
to a condition driven by aberrant wound healing following alveolar epithelial 
cell injury that results in scarring of the lung, architectural distortion, and 
irreversible loss of function. Improved understanding of disease pathogenesis 
has led to the identification of several therapeutic targets and the design of 
high-quality clinical trials evaluating novel compounds. However, the results of 
these studies have been mostly disappointing, probably due to the plethora of 
mediators, growth factors, and signaling pathways involved in the fibrotic 
process. Most recently, pirfenidone and nintedanib, two compounds with 
pleiotropic anti-fibrotic properties, have been proven effective in reducing 
functional decline and disease progression in IPF. This is a major breakthrough. 
Nevertheless, we still have a long way to go. In fact, neither pirfenidone nor 
nintedanib is a cure for IPF, and most patients continue to progress despite 
treatment. As such, comprehensive care of patients with IPF, including 
management of concomitant conditions and physical debility, as well as timely 
referral for lung transplantation, remains essential. Several agents with a high 
potential are currently being tested, and many more are ready for clinical 
trials. Their completion is critical for achieving the ultimate goal of curing 
patients with IPF.